Concomitant methotrexate boosts pegloticase efficacy in gout patients

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Coadministration of oral methotrexate dramatically improves the effectiveness and durability of response to pegloticase (Krystexxa) in patients with uncontrolled refractory tophaceous gout, Orrin M. Troum, MD, said at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Alvin F. Wells (left) and Dr. Orrin M. Troum
Bruce Jancin/MDedge News
Dr. Alvin F. Wells (left) and Dr. Orrin M. Troum

He cited what he considers to be a practice-changing, prospective, observational, proof-of-concept study presented by John Botson, MD, at the 2018 annual meeting of the American College of Rheumatology.

Dr. Botson, a rheumatologist at Orthopedic Physicians Alaska, in Anchorage, reported on nine patients with refractory tophaceous gout placed on an 8-mg infusion of pegloticase every 2 weeks as third-line therapy. But 1 month beforehand he put them on oral methotrexate at 15 mg once weekly along with folic acid at 1 mg/day in an effort to prevent the development of treatment-limiting anti-pegloticase antibodies. It’s the same strategy rheumatologists often use when patients with rheumatoid arthritis on a tumor necrosis factor inhibitor begin to develop anti-drug antibodies.

At the time of the ACR meeting, all nine patients had received at least nine infusions, and six had received at least 12 infusions over the course of 6 months. The response rate was 100%, defined as more than 80% of serum uric acid levels being below 6.0 mg/dL. All patients stayed on methotrexate with no dose adjustment. And there were no infusion reactions. In contrast, the response rate in the randomized trials of pegloticase was only 42%, and 26% of pegloticase recipients experienced infusion reactions within 6 months.

“Although this is not [Food and Drug Administration] approved, it makes a lot of sense. From my standpoint, this is something that I’m doing now for my patients starting on pegloticase if there’s no contraindication to using methotrexate,” said Dr. Troum, a rheumatologist at the University of Southern California in Los Angeles.

“I’ve been doing this, too. This really did change my practice,” added his fellow panelist Alvin F. Wells, MD, PhD, director of the Rheumatology and Immunotherapy Center in Franklin, Wisc.

When they asked for a show of hands, only a handful of audience members indicated they are now using methotrexate in conjunction with pegloticase in their tophaceous gout patients.



Dr. Wells said his sole reservation about the practice involves using methotrexate in patients with an elevated creatinine level. What about using azathioprine or corticosteroids instead? he asked.

Dr. Troum replied that he monitors those patients carefully but sticks with the methotrexate because it’s only for a few months, which is the time frame in which patients are especially vulnerable to experiencing loss of response to pegloticase due to development of anti-drug antibodies.

Dr. Botson, who was in the Maui audience, rose to give a study update. With additional follow-up, he said, there has still been no signal of loss of response to pegloticase coadministered with methotrexate.

“A lot of us are starting to feel like immunosuppression, whether it’s with methotrexate or something else, is standard of care now,” according to the rheumatologist.

As to prescribing methotrexate in gout patients with renal insufficiency, he continued, he and his colleagues have given the matter quite a bit of thought.

“You’re talking about using methotrexate for 6 months in most of these cases. A lot of the patients who have really bad tophaceous gout already have renal insufficiency, and in the short term we haven’t really seen any problems with that. We work closely with a nephrologist on those cases. And a lot of nephrologists swear – although I don’t think the data are there – that they actually improve their renal function when we start to treat their tophaceous gout,” Dr. Botson said.

Dr. Troum and Dr. Wells reported serving as consultants to and on speakers bureaus for numerous pharmaceutical companies.

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Coadministration of oral methotrexate dramatically improves the effectiveness and durability of response to pegloticase (Krystexxa) in patients with uncontrolled refractory tophaceous gout, Orrin M. Troum, MD, said at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Alvin F. Wells (left) and Dr. Orrin M. Troum
Bruce Jancin/MDedge News
Dr. Alvin F. Wells (left) and Dr. Orrin M. Troum

He cited what he considers to be a practice-changing, prospective, observational, proof-of-concept study presented by John Botson, MD, at the 2018 annual meeting of the American College of Rheumatology.

Dr. Botson, a rheumatologist at Orthopedic Physicians Alaska, in Anchorage, reported on nine patients with refractory tophaceous gout placed on an 8-mg infusion of pegloticase every 2 weeks as third-line therapy. But 1 month beforehand he put them on oral methotrexate at 15 mg once weekly along with folic acid at 1 mg/day in an effort to prevent the development of treatment-limiting anti-pegloticase antibodies. It’s the same strategy rheumatologists often use when patients with rheumatoid arthritis on a tumor necrosis factor inhibitor begin to develop anti-drug antibodies.

At the time of the ACR meeting, all nine patients had received at least nine infusions, and six had received at least 12 infusions over the course of 6 months. The response rate was 100%, defined as more than 80% of serum uric acid levels being below 6.0 mg/dL. All patients stayed on methotrexate with no dose adjustment. And there were no infusion reactions. In contrast, the response rate in the randomized trials of pegloticase was only 42%, and 26% of pegloticase recipients experienced infusion reactions within 6 months.

“Although this is not [Food and Drug Administration] approved, it makes a lot of sense. From my standpoint, this is something that I’m doing now for my patients starting on pegloticase if there’s no contraindication to using methotrexate,” said Dr. Troum, a rheumatologist at the University of Southern California in Los Angeles.

“I’ve been doing this, too. This really did change my practice,” added his fellow panelist Alvin F. Wells, MD, PhD, director of the Rheumatology and Immunotherapy Center in Franklin, Wisc.

When they asked for a show of hands, only a handful of audience members indicated they are now using methotrexate in conjunction with pegloticase in their tophaceous gout patients.



Dr. Wells said his sole reservation about the practice involves using methotrexate in patients with an elevated creatinine level. What about using azathioprine or corticosteroids instead? he asked.

Dr. Troum replied that he monitors those patients carefully but sticks with the methotrexate because it’s only for a few months, which is the time frame in which patients are especially vulnerable to experiencing loss of response to pegloticase due to development of anti-drug antibodies.

Dr. Botson, who was in the Maui audience, rose to give a study update. With additional follow-up, he said, there has still been no signal of loss of response to pegloticase coadministered with methotrexate.

“A lot of us are starting to feel like immunosuppression, whether it’s with methotrexate or something else, is standard of care now,” according to the rheumatologist.

As to prescribing methotrexate in gout patients with renal insufficiency, he continued, he and his colleagues have given the matter quite a bit of thought.

“You’re talking about using methotrexate for 6 months in most of these cases. A lot of the patients who have really bad tophaceous gout already have renal insufficiency, and in the short term we haven’t really seen any problems with that. We work closely with a nephrologist on those cases. And a lot of nephrologists swear – although I don’t think the data are there – that they actually improve their renal function when we start to treat their tophaceous gout,” Dr. Botson said.

Dr. Troum and Dr. Wells reported serving as consultants to and on speakers bureaus for numerous pharmaceutical companies.

 

Coadministration of oral methotrexate dramatically improves the effectiveness and durability of response to pegloticase (Krystexxa) in patients with uncontrolled refractory tophaceous gout, Orrin M. Troum, MD, said at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Alvin F. Wells (left) and Dr. Orrin M. Troum
Bruce Jancin/MDedge News
Dr. Alvin F. Wells (left) and Dr. Orrin M. Troum

He cited what he considers to be a practice-changing, prospective, observational, proof-of-concept study presented by John Botson, MD, at the 2018 annual meeting of the American College of Rheumatology.

Dr. Botson, a rheumatologist at Orthopedic Physicians Alaska, in Anchorage, reported on nine patients with refractory tophaceous gout placed on an 8-mg infusion of pegloticase every 2 weeks as third-line therapy. But 1 month beforehand he put them on oral methotrexate at 15 mg once weekly along with folic acid at 1 mg/day in an effort to prevent the development of treatment-limiting anti-pegloticase antibodies. It’s the same strategy rheumatologists often use when patients with rheumatoid arthritis on a tumor necrosis factor inhibitor begin to develop anti-drug antibodies.

At the time of the ACR meeting, all nine patients had received at least nine infusions, and six had received at least 12 infusions over the course of 6 months. The response rate was 100%, defined as more than 80% of serum uric acid levels being below 6.0 mg/dL. All patients stayed on methotrexate with no dose adjustment. And there were no infusion reactions. In contrast, the response rate in the randomized trials of pegloticase was only 42%, and 26% of pegloticase recipients experienced infusion reactions within 6 months.

“Although this is not [Food and Drug Administration] approved, it makes a lot of sense. From my standpoint, this is something that I’m doing now for my patients starting on pegloticase if there’s no contraindication to using methotrexate,” said Dr. Troum, a rheumatologist at the University of Southern California in Los Angeles.

“I’ve been doing this, too. This really did change my practice,” added his fellow panelist Alvin F. Wells, MD, PhD, director of the Rheumatology and Immunotherapy Center in Franklin, Wisc.

When they asked for a show of hands, only a handful of audience members indicated they are now using methotrexate in conjunction with pegloticase in their tophaceous gout patients.



Dr. Wells said his sole reservation about the practice involves using methotrexate in patients with an elevated creatinine level. What about using azathioprine or corticosteroids instead? he asked.

Dr. Troum replied that he monitors those patients carefully but sticks with the methotrexate because it’s only for a few months, which is the time frame in which patients are especially vulnerable to experiencing loss of response to pegloticase due to development of anti-drug antibodies.

Dr. Botson, who was in the Maui audience, rose to give a study update. With additional follow-up, he said, there has still been no signal of loss of response to pegloticase coadministered with methotrexate.

“A lot of us are starting to feel like immunosuppression, whether it’s with methotrexate or something else, is standard of care now,” according to the rheumatologist.

As to prescribing methotrexate in gout patients with renal insufficiency, he continued, he and his colleagues have given the matter quite a bit of thought.

“You’re talking about using methotrexate for 6 months in most of these cases. A lot of the patients who have really bad tophaceous gout already have renal insufficiency, and in the short term we haven’t really seen any problems with that. We work closely with a nephrologist on those cases. And a lot of nephrologists swear – although I don’t think the data are there – that they actually improve their renal function when we start to treat their tophaceous gout,” Dr. Botson said.

Dr. Troum and Dr. Wells reported serving as consultants to and on speakers bureaus for numerous pharmaceutical companies.

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Recent trials advance axial spondyloarthritis therapy

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Changed
Wed, 03/13/2019 - 15:35

 

– Arguably the most exciting therapeutic development in axial spondyloarthritis in the past year was the demonstrated efficacy and safety of the investigational oral selective Janus kinase 1 inhibitor filgotinib in the setting of active ankylosing spondylitis, speakers agreed at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Eric Ruderman (left) and Dr. Arthur Kavanaugh
Bruce Jancin/MDedge News
Dr. Eric Ruderman (left) and Dr. Arthur Kavanaugh

“This is big, big news,” commented symposium director Arthur Kavanaugh, MD. “This is going to be a big deal.”

Other recent clinical trials of note in axial spondyloarthritis (SpA) highlighted by Dr. Kavanaugh and Eric Ruderman, MD, professor of medicine at Northwestern University, Chicago, included a positive phase 3 study of certolizumab pegol (Cimzia) in nonradiographic SpA, two positive phase 3 trials of the interleukin-17A antagonist ixekizumab (Taltz) in radiographic SpA, a positive phase 2b trial of the dual IL-17A/F antagonist bimekizumab, and publication of three surprisingly negative phase 3 trials of the IL-12/23 inhibitor ustekinumab (Stelara).

Filgotinib

TORTUGA was a phase 2b, double-blind, multicenter trial of 116 European patients with active ankylosing spondylitis nonresponsive to NSAIDs who were randomized to oral filgotinib at 200 mg once daily or placebo for 12 weeks. Filgotinib reduced the Ankylosing Spondylitis Disease Activity Score (ASDAS) by a mean of 1.47 points from baseline, a significantly better result for the primary outcome than the 0.57-point decrease in controls (Lancet. 2018 Dec 1;392[10162]:2378-87).

Dr. Ruderman was also favorably impressed with the oral Janus kinase 1 (JAK1) inhibitor’s performance on the secondary outcome measures, including a mean 2.41-point reduction from baseline on the Bath Ankylosing Spondylitis Disease Activity Index, compared with a 1.44-point decrease in controls, with the difference being significant from week 8 onward. The filgotinib group also did significantly better on validated measures of physical function, spinal mobility, physical function, quality of life, peripheral arthritis, fatigue, and spinal and sacroiliac joint inflammation as assessed by MRI.

One patient in the filgotinib group, a smoker, developed pneumonia and another experienced deep venous thrombosis.

The study results are an exciting development because SpA treatments with new mechanisms of action are sorely needed. NSAIDs are considered first-line pharmacotherapy at present, with various tumor necrosis factor (TNF) inhibitors as well as the IL-17 inhibitor secukinumab (Cosentyx) the only approved biologic alternatives.

“This is the most impressive data I’ve seen that JAK inhibitors are effective in ankylosing spondyloarthritis,” commented Paul Emery, MD, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

TORTUGA was the first positive phase 2 trial of a selective JAK1 inhibitor in SpA. However, Dr. Kavanaugh noted that while a phase 2 trial of tofacitinib (Xeljanz) failed to meet its “very convoluted” primary endpoint, the JAK1/3 inhibitor was positive for key secondary endpoints, including favorable MRI changes. And a phase 3 trial of tofacitinib in SpA is underway.

A key remaining question pending the outcome of definitive phase 3 trials is whether specificity of JAK enzyme inhibition matters or if a class effect is at work, according to Dr. Kavanaugh, professor of medicine at the University of California, San Diego.

 

 

Certolizumab pegol

The TNF inhibitor is already approved for ankylosing spondylitis, among other indications, but it has now demonstrated efficacy and safety in nonradiographic SpA in a phase 3 trial structured with guidance from the Food and Drug Administration.

“It seems likely that UCB will get the indication for this,” according to Dr. Ruderman.

This 317-patient trial was remarkable in that it entailed a full 52 weeks of double-blind therapy with certolizumab at the standard dose of 200 mg every 2 weeks or placebo. The ASDAS Major Improvement rate, defined as at least a 2-point improvement from baseline, was 47% in the active treatment arm, compared with 7% on placebo. The Assessment in Ankylosing Spondylitis International Society 40% (ASAS 40) response rate, a more patient-reported outcome measure, was 57% in the certolizumab group and 16% in controls in this trial, which was recently published (Arthritis Rheumatol. 2019 March 8. doi: 10.1002/art.40866). All participants had to have baseline MRI evidence of sacroiliac joint inflammation and/or an elevated C-reactive protein.

By way of background, Dr. Ruderman explained that the FDA required 52 weeks of double-blind, placebo-controlled therapy because the agency’s advisory committee had formerly expressed reservations about considering an expanded indication for TNF inhibitors in nonradiographic as opposed to radiographic SpA.



“They were very concerned that approval could result in patients with mechanical back pain or fibromyalgia being treated with biologics. And they weren’t sure nonradiographic SpA was a discrete entity. They wondered if it remits on its own,” according to the rheumatologist.

Dr. Ruderman is curious to see how the FDA is going to handle this situation in light of the positive phase 3 certolizumab results. Will the agency require other companies that market TNF inhibitors to mount a similarly rigorous 52-week, double-blind, placebo-controlled trial in order to obtain an expanded indication? That would seem to pose ethical issues now. Or will the companies be able to gain an expanded indication by retrospective analysis of outcomes in patients with nonradiographic SpA in their existing trials databases? Stay tuned.

Ixekizumab

The COAST-V trial was a phase 3, randomized, double-blind, active- and placebo-controlled trial of 341 patients with radiographic SpA who hadn’t previously been treated with a biologic disease-modifying antirheumatic drug. They were assigned to 80 mg of ixekizumab every 2 weeks, 80 mg every 4 weeks, adalimumab (Humira) at 40 mg every 2 weeks, or placebo. The primary endpoint – an ASAS 40 response at week 16 – was achieved in 52% of patients on ixekizumab every 2 weeks, 48% of those who received ixekizumab every 4 weeks, 36% on adalimumab, and 18% on placebo (Lancet. 2018 Dec 8;392[10163]:2441-51).

In contrast, the phase 3 COAST-W trial included 316 patients with radiographic SpA who were inadequate responders or intolerant to one or more prior anti-TNF agents. The 16-week ASAS 40 response rate was 30.6% with ixekizumab every 2 weeks, similar at 25.4% with ixekizumab every 4 weeks, and 12.5% with placebo (Arthritis Rheumatol. 2018 Oct 20. doi: 10.1002/art.40753).

While the COAST-V trial convincingly showed both ixekizumab and adalimumab were more effective than placebo, the patient numbers were way too small to draw any conclusions about the relative efficacy of the two biologics, according to Dr. Ruderman.

“I don’t think these results are surprising,” Dr. Kavanaugh commented. “It would have been surprising if ixekizumab was ineffective, given that secukinumab works. But it’s nice to have the proof.”

 

 

Bimekizumab

This investigational dual IL-17A/F inhibitor demonstrated efficacy and safety for SpA in a 297-patient, phase 2b trial presented at the 2018 European Congress of Rheumatology.

“It’s effective, but it doesn’t look like it’s particularly more effective than either of the existing IL-17A inhibitors. We’ll see going forward if there truly is an advantage here to the additional inhibition of IL-17F in this population. I will say that the preclinical and laboratory data on the potential advantages of IL-17F inhibition are mostly in the psoriasis/psoriatic arthritis space. It’s not clear in ankylosing spondyloarthritis specifically whether we should expect to see a difference,” Dr. Ruderman said.

Ustekinumab

The IL-12/23 inhibitor proved no better than placebo in patients with SpA in three separate phase 3, randomized trials recently published as a single summary article (Arthritis Rheumatol. 2019 Feb;71[2]:258-70).

“Ustekinumab was effective in an earlier open study, so I think everybody was surprised by this,” Dr. Kavanaugh said.

He reported serving as a consultant to and/or receiving research funding from a dozen pharmaceutical companies. Dr. Ruderman reported financial relationships with eight companies.

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– Arguably the most exciting therapeutic development in axial spondyloarthritis in the past year was the demonstrated efficacy and safety of the investigational oral selective Janus kinase 1 inhibitor filgotinib in the setting of active ankylosing spondylitis, speakers agreed at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Eric Ruderman (left) and Dr. Arthur Kavanaugh
Bruce Jancin/MDedge News
Dr. Eric Ruderman (left) and Dr. Arthur Kavanaugh

“This is big, big news,” commented symposium director Arthur Kavanaugh, MD. “This is going to be a big deal.”

Other recent clinical trials of note in axial spondyloarthritis (SpA) highlighted by Dr. Kavanaugh and Eric Ruderman, MD, professor of medicine at Northwestern University, Chicago, included a positive phase 3 study of certolizumab pegol (Cimzia) in nonradiographic SpA, two positive phase 3 trials of the interleukin-17A antagonist ixekizumab (Taltz) in radiographic SpA, a positive phase 2b trial of the dual IL-17A/F antagonist bimekizumab, and publication of three surprisingly negative phase 3 trials of the IL-12/23 inhibitor ustekinumab (Stelara).

Filgotinib

TORTUGA was a phase 2b, double-blind, multicenter trial of 116 European patients with active ankylosing spondylitis nonresponsive to NSAIDs who were randomized to oral filgotinib at 200 mg once daily or placebo for 12 weeks. Filgotinib reduced the Ankylosing Spondylitis Disease Activity Score (ASDAS) by a mean of 1.47 points from baseline, a significantly better result for the primary outcome than the 0.57-point decrease in controls (Lancet. 2018 Dec 1;392[10162]:2378-87).

Dr. Ruderman was also favorably impressed with the oral Janus kinase 1 (JAK1) inhibitor’s performance on the secondary outcome measures, including a mean 2.41-point reduction from baseline on the Bath Ankylosing Spondylitis Disease Activity Index, compared with a 1.44-point decrease in controls, with the difference being significant from week 8 onward. The filgotinib group also did significantly better on validated measures of physical function, spinal mobility, physical function, quality of life, peripheral arthritis, fatigue, and spinal and sacroiliac joint inflammation as assessed by MRI.

One patient in the filgotinib group, a smoker, developed pneumonia and another experienced deep venous thrombosis.

The study results are an exciting development because SpA treatments with new mechanisms of action are sorely needed. NSAIDs are considered first-line pharmacotherapy at present, with various tumor necrosis factor (TNF) inhibitors as well as the IL-17 inhibitor secukinumab (Cosentyx) the only approved biologic alternatives.

“This is the most impressive data I’ve seen that JAK inhibitors are effective in ankylosing spondyloarthritis,” commented Paul Emery, MD, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

TORTUGA was the first positive phase 2 trial of a selective JAK1 inhibitor in SpA. However, Dr. Kavanaugh noted that while a phase 2 trial of tofacitinib (Xeljanz) failed to meet its “very convoluted” primary endpoint, the JAK1/3 inhibitor was positive for key secondary endpoints, including favorable MRI changes. And a phase 3 trial of tofacitinib in SpA is underway.

A key remaining question pending the outcome of definitive phase 3 trials is whether specificity of JAK enzyme inhibition matters or if a class effect is at work, according to Dr. Kavanaugh, professor of medicine at the University of California, San Diego.

 

 

Certolizumab pegol

The TNF inhibitor is already approved for ankylosing spondylitis, among other indications, but it has now demonstrated efficacy and safety in nonradiographic SpA in a phase 3 trial structured with guidance from the Food and Drug Administration.

“It seems likely that UCB will get the indication for this,” according to Dr. Ruderman.

This 317-patient trial was remarkable in that it entailed a full 52 weeks of double-blind therapy with certolizumab at the standard dose of 200 mg every 2 weeks or placebo. The ASDAS Major Improvement rate, defined as at least a 2-point improvement from baseline, was 47% in the active treatment arm, compared with 7% on placebo. The Assessment in Ankylosing Spondylitis International Society 40% (ASAS 40) response rate, a more patient-reported outcome measure, was 57% in the certolizumab group and 16% in controls in this trial, which was recently published (Arthritis Rheumatol. 2019 March 8. doi: 10.1002/art.40866). All participants had to have baseline MRI evidence of sacroiliac joint inflammation and/or an elevated C-reactive protein.

By way of background, Dr. Ruderman explained that the FDA required 52 weeks of double-blind, placebo-controlled therapy because the agency’s advisory committee had formerly expressed reservations about considering an expanded indication for TNF inhibitors in nonradiographic as opposed to radiographic SpA.



“They were very concerned that approval could result in patients with mechanical back pain or fibromyalgia being treated with biologics. And they weren’t sure nonradiographic SpA was a discrete entity. They wondered if it remits on its own,” according to the rheumatologist.

Dr. Ruderman is curious to see how the FDA is going to handle this situation in light of the positive phase 3 certolizumab results. Will the agency require other companies that market TNF inhibitors to mount a similarly rigorous 52-week, double-blind, placebo-controlled trial in order to obtain an expanded indication? That would seem to pose ethical issues now. Or will the companies be able to gain an expanded indication by retrospective analysis of outcomes in patients with nonradiographic SpA in their existing trials databases? Stay tuned.

Ixekizumab

The COAST-V trial was a phase 3, randomized, double-blind, active- and placebo-controlled trial of 341 patients with radiographic SpA who hadn’t previously been treated with a biologic disease-modifying antirheumatic drug. They were assigned to 80 mg of ixekizumab every 2 weeks, 80 mg every 4 weeks, adalimumab (Humira) at 40 mg every 2 weeks, or placebo. The primary endpoint – an ASAS 40 response at week 16 – was achieved in 52% of patients on ixekizumab every 2 weeks, 48% of those who received ixekizumab every 4 weeks, 36% on adalimumab, and 18% on placebo (Lancet. 2018 Dec 8;392[10163]:2441-51).

In contrast, the phase 3 COAST-W trial included 316 patients with radiographic SpA who were inadequate responders or intolerant to one or more prior anti-TNF agents. The 16-week ASAS 40 response rate was 30.6% with ixekizumab every 2 weeks, similar at 25.4% with ixekizumab every 4 weeks, and 12.5% with placebo (Arthritis Rheumatol. 2018 Oct 20. doi: 10.1002/art.40753).

While the COAST-V trial convincingly showed both ixekizumab and adalimumab were more effective than placebo, the patient numbers were way too small to draw any conclusions about the relative efficacy of the two biologics, according to Dr. Ruderman.

“I don’t think these results are surprising,” Dr. Kavanaugh commented. “It would have been surprising if ixekizumab was ineffective, given that secukinumab works. But it’s nice to have the proof.”

 

 

Bimekizumab

This investigational dual IL-17A/F inhibitor demonstrated efficacy and safety for SpA in a 297-patient, phase 2b trial presented at the 2018 European Congress of Rheumatology.

“It’s effective, but it doesn’t look like it’s particularly more effective than either of the existing IL-17A inhibitors. We’ll see going forward if there truly is an advantage here to the additional inhibition of IL-17F in this population. I will say that the preclinical and laboratory data on the potential advantages of IL-17F inhibition are mostly in the psoriasis/psoriatic arthritis space. It’s not clear in ankylosing spondyloarthritis specifically whether we should expect to see a difference,” Dr. Ruderman said.

Ustekinumab

The IL-12/23 inhibitor proved no better than placebo in patients with SpA in three separate phase 3, randomized trials recently published as a single summary article (Arthritis Rheumatol. 2019 Feb;71[2]:258-70).

“Ustekinumab was effective in an earlier open study, so I think everybody was surprised by this,” Dr. Kavanaugh said.

He reported serving as a consultant to and/or receiving research funding from a dozen pharmaceutical companies. Dr. Ruderman reported financial relationships with eight companies.

 

– Arguably the most exciting therapeutic development in axial spondyloarthritis in the past year was the demonstrated efficacy and safety of the investigational oral selective Janus kinase 1 inhibitor filgotinib in the setting of active ankylosing spondylitis, speakers agreed at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Eric Ruderman (left) and Dr. Arthur Kavanaugh
Bruce Jancin/MDedge News
Dr. Eric Ruderman (left) and Dr. Arthur Kavanaugh

“This is big, big news,” commented symposium director Arthur Kavanaugh, MD. “This is going to be a big deal.”

Other recent clinical trials of note in axial spondyloarthritis (SpA) highlighted by Dr. Kavanaugh and Eric Ruderman, MD, professor of medicine at Northwestern University, Chicago, included a positive phase 3 study of certolizumab pegol (Cimzia) in nonradiographic SpA, two positive phase 3 trials of the interleukin-17A antagonist ixekizumab (Taltz) in radiographic SpA, a positive phase 2b trial of the dual IL-17A/F antagonist bimekizumab, and publication of three surprisingly negative phase 3 trials of the IL-12/23 inhibitor ustekinumab (Stelara).

Filgotinib

TORTUGA was a phase 2b, double-blind, multicenter trial of 116 European patients with active ankylosing spondylitis nonresponsive to NSAIDs who were randomized to oral filgotinib at 200 mg once daily or placebo for 12 weeks. Filgotinib reduced the Ankylosing Spondylitis Disease Activity Score (ASDAS) by a mean of 1.47 points from baseline, a significantly better result for the primary outcome than the 0.57-point decrease in controls (Lancet. 2018 Dec 1;392[10162]:2378-87).

Dr. Ruderman was also favorably impressed with the oral Janus kinase 1 (JAK1) inhibitor’s performance on the secondary outcome measures, including a mean 2.41-point reduction from baseline on the Bath Ankylosing Spondylitis Disease Activity Index, compared with a 1.44-point decrease in controls, with the difference being significant from week 8 onward. The filgotinib group also did significantly better on validated measures of physical function, spinal mobility, physical function, quality of life, peripheral arthritis, fatigue, and spinal and sacroiliac joint inflammation as assessed by MRI.

One patient in the filgotinib group, a smoker, developed pneumonia and another experienced deep venous thrombosis.

The study results are an exciting development because SpA treatments with new mechanisms of action are sorely needed. NSAIDs are considered first-line pharmacotherapy at present, with various tumor necrosis factor (TNF) inhibitors as well as the IL-17 inhibitor secukinumab (Cosentyx) the only approved biologic alternatives.

“This is the most impressive data I’ve seen that JAK inhibitors are effective in ankylosing spondyloarthritis,” commented Paul Emery, MD, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

TORTUGA was the first positive phase 2 trial of a selective JAK1 inhibitor in SpA. However, Dr. Kavanaugh noted that while a phase 2 trial of tofacitinib (Xeljanz) failed to meet its “very convoluted” primary endpoint, the JAK1/3 inhibitor was positive for key secondary endpoints, including favorable MRI changes. And a phase 3 trial of tofacitinib in SpA is underway.

A key remaining question pending the outcome of definitive phase 3 trials is whether specificity of JAK enzyme inhibition matters or if a class effect is at work, according to Dr. Kavanaugh, professor of medicine at the University of California, San Diego.

 

 

Certolizumab pegol

The TNF inhibitor is already approved for ankylosing spondylitis, among other indications, but it has now demonstrated efficacy and safety in nonradiographic SpA in a phase 3 trial structured with guidance from the Food and Drug Administration.

“It seems likely that UCB will get the indication for this,” according to Dr. Ruderman.

This 317-patient trial was remarkable in that it entailed a full 52 weeks of double-blind therapy with certolizumab at the standard dose of 200 mg every 2 weeks or placebo. The ASDAS Major Improvement rate, defined as at least a 2-point improvement from baseline, was 47% in the active treatment arm, compared with 7% on placebo. The Assessment in Ankylosing Spondylitis International Society 40% (ASAS 40) response rate, a more patient-reported outcome measure, was 57% in the certolizumab group and 16% in controls in this trial, which was recently published (Arthritis Rheumatol. 2019 March 8. doi: 10.1002/art.40866). All participants had to have baseline MRI evidence of sacroiliac joint inflammation and/or an elevated C-reactive protein.

By way of background, Dr. Ruderman explained that the FDA required 52 weeks of double-blind, placebo-controlled therapy because the agency’s advisory committee had formerly expressed reservations about considering an expanded indication for TNF inhibitors in nonradiographic as opposed to radiographic SpA.



“They were very concerned that approval could result in patients with mechanical back pain or fibromyalgia being treated with biologics. And they weren’t sure nonradiographic SpA was a discrete entity. They wondered if it remits on its own,” according to the rheumatologist.

Dr. Ruderman is curious to see how the FDA is going to handle this situation in light of the positive phase 3 certolizumab results. Will the agency require other companies that market TNF inhibitors to mount a similarly rigorous 52-week, double-blind, placebo-controlled trial in order to obtain an expanded indication? That would seem to pose ethical issues now. Or will the companies be able to gain an expanded indication by retrospective analysis of outcomes in patients with nonradiographic SpA in their existing trials databases? Stay tuned.

Ixekizumab

The COAST-V trial was a phase 3, randomized, double-blind, active- and placebo-controlled trial of 341 patients with radiographic SpA who hadn’t previously been treated with a biologic disease-modifying antirheumatic drug. They were assigned to 80 mg of ixekizumab every 2 weeks, 80 mg every 4 weeks, adalimumab (Humira) at 40 mg every 2 weeks, or placebo. The primary endpoint – an ASAS 40 response at week 16 – was achieved in 52% of patients on ixekizumab every 2 weeks, 48% of those who received ixekizumab every 4 weeks, 36% on adalimumab, and 18% on placebo (Lancet. 2018 Dec 8;392[10163]:2441-51).

In contrast, the phase 3 COAST-W trial included 316 patients with radiographic SpA who were inadequate responders or intolerant to one or more prior anti-TNF agents. The 16-week ASAS 40 response rate was 30.6% with ixekizumab every 2 weeks, similar at 25.4% with ixekizumab every 4 weeks, and 12.5% with placebo (Arthritis Rheumatol. 2018 Oct 20. doi: 10.1002/art.40753).

While the COAST-V trial convincingly showed both ixekizumab and adalimumab were more effective than placebo, the patient numbers were way too small to draw any conclusions about the relative efficacy of the two biologics, according to Dr. Ruderman.

“I don’t think these results are surprising,” Dr. Kavanaugh commented. “It would have been surprising if ixekizumab was ineffective, given that secukinumab works. But it’s nice to have the proof.”

 

 

Bimekizumab

This investigational dual IL-17A/F inhibitor demonstrated efficacy and safety for SpA in a 297-patient, phase 2b trial presented at the 2018 European Congress of Rheumatology.

“It’s effective, but it doesn’t look like it’s particularly more effective than either of the existing IL-17A inhibitors. We’ll see going forward if there truly is an advantage here to the additional inhibition of IL-17F in this population. I will say that the preclinical and laboratory data on the potential advantages of IL-17F inhibition are mostly in the psoriasis/psoriatic arthritis space. It’s not clear in ankylosing spondyloarthritis specifically whether we should expect to see a difference,” Dr. Ruderman said.

Ustekinumab

The IL-12/23 inhibitor proved no better than placebo in patients with SpA in three separate phase 3, randomized trials recently published as a single summary article (Arthritis Rheumatol. 2019 Feb;71[2]:258-70).

“Ustekinumab was effective in an earlier open study, so I think everybody was surprised by this,” Dr. Kavanaugh said.

He reported serving as a consultant to and/or receiving research funding from a dozen pharmaceutical companies. Dr. Ruderman reported financial relationships with eight companies.

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Novel RA strategy: Target first-line biologics for likely methotrexate nonresponders

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Mon, 03/25/2019 - 10:50

 

– An ongoing proof-of-concept study in the United Kingdom uses T-cell subset analysis to identify those patients with early rheumatoid arthritis who are unlikely to experience remission with methotrexate alone and therefore warrant more potent first-line therapy with a tumor necrosis factor (TNF) inhibitor in combination with methotrexate, Paul Emery, MD, said at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Paul Emery, University of Leeds, England
Bruce Jancin/MDedge News
Dr. Paul Emery

“When we get the readout from this study, if we get the results we expect, we could actually put it to the National Health Service that it would be cost saving to use biologics as first-line therapy in a proportion, which will be about 40% of patients. But we won’t necessarily need to use them for a year, and we’ll get a 70%-plus remission rate, which I think is the sort of level we should be asking for in our patients,” according to Dr. Emery, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

This proof-of-concept study capitalizes on earlier work by Dr. Emery and coinvestigators, who showed in 70 patients with early rheumatoid arthritis given methotrexate as their first-ever disease-modifying antirheumatic drug (DMARD) that those with a normal pretreatment frequency of naive CD4+ T cells had an 83% remission rate at 6 months as defined by a Disease Activity Score in 28 joints (DAS28) of less than 2.6. In contrast, only 21% of those with a reduced naive CD4+ T-cell frequency experienced remission when compared with healthy controls. In an analysis adjusted for age and the presence of anti–citrullinated protein antibodies (ACPA), a normal baseline naive CD4+ T-cell frequency was associated with a 5.9-fold increased likelihood of remission on methotrexate (Ann Rheum Dis. 2014 Nov;73[11]:2047-53).

In the new proof-of-concept study, DMARD-naive, ACPA-positive patients with early RA undergo T-cell subset quantification by flow cytometry. Thirty patients with a normal test result are assigned to methotrexate with treat-to-target dose escalation. Based upon the investigators’ earlier work, it’s anticipated that about 80% of these patients will be in remission at 6 months.

Sixty patients with an abnormal baseline T-cell test result are being randomized to methotrexate plus either placebo or biosimilar etanercept. Again based upon the earlier study, the expected remission rate at 6 months in the methotrexate-plus-placebo group is about 20%. In contrast, the anticipated remission rate in the patients on an anti-TNF biologic plus methotrexate as first-line therapy is about 70% on the basis of the results of previous clinical trials, including PRIZE (N Engl J Med. 2014 Nov 6;371[19]:1781-92) and COMET (Ann Rheum Dis. 2012 Jun;71[6]:989-92).

Meanwhile, the price tag for biosimilar TNF inhibitors in the United Kingdom has come down to the point that routine across-the-board use of biologics as first-line therapy is arguably cost effective, a situation Dr. Emery described as hitherto “the unthinkable.”



The cost of biosimilar adalimumab in the coming year will be less than $3,000 annually in the U.K. health care system. So if 100 patients with early RA and no contraindication to biologic therapy are placed on biosimilar adalimumab and methotrexate for 1 year, the total cost for the biologic in this cohort will be less than $300,000, and roughly 70 of the 100 patients will have achieved remission. This approach makes much more sense than current standard practice, which is to reserve biologics as second-line therapy for patients who have failed to achieve remission on nonbiologic DMARDs, thereby allowing their joint damage to advance in the interim to the point that they need to stay on biologic therapy for decades, the rheumatologist argued.

“Once one accepts that remission has become the aim of therapy, then the facts, I think, speak for themselves: There’s absolutely no doubt that the rate of remission is best with the first DMARD. So if our aim is remission, we should use that one opportunity with the best agent first, because we’re not going to get the same response later,” Dr. Emery said.

Also, “there’s no doubt” that the dose of biologics can be halved with no loss of efficacy in patients who achieve remission on full-dose therapy, as previously demonstrated in PRIZE and other trials. This strategy further reduces the overall cost of biologic therapy, he added.

Dr. Emery, who recently received the Order of the British Empire from Queen Elizabeth personally in recognition of his career achievements in rheumatology, reported having no financial conflicts of interest regarding his presentation.

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– An ongoing proof-of-concept study in the United Kingdom uses T-cell subset analysis to identify those patients with early rheumatoid arthritis who are unlikely to experience remission with methotrexate alone and therefore warrant more potent first-line therapy with a tumor necrosis factor (TNF) inhibitor in combination with methotrexate, Paul Emery, MD, said at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Paul Emery, University of Leeds, England
Bruce Jancin/MDedge News
Dr. Paul Emery

“When we get the readout from this study, if we get the results we expect, we could actually put it to the National Health Service that it would be cost saving to use biologics as first-line therapy in a proportion, which will be about 40% of patients. But we won’t necessarily need to use them for a year, and we’ll get a 70%-plus remission rate, which I think is the sort of level we should be asking for in our patients,” according to Dr. Emery, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

This proof-of-concept study capitalizes on earlier work by Dr. Emery and coinvestigators, who showed in 70 patients with early rheumatoid arthritis given methotrexate as their first-ever disease-modifying antirheumatic drug (DMARD) that those with a normal pretreatment frequency of naive CD4+ T cells had an 83% remission rate at 6 months as defined by a Disease Activity Score in 28 joints (DAS28) of less than 2.6. In contrast, only 21% of those with a reduced naive CD4+ T-cell frequency experienced remission when compared with healthy controls. In an analysis adjusted for age and the presence of anti–citrullinated protein antibodies (ACPA), a normal baseline naive CD4+ T-cell frequency was associated with a 5.9-fold increased likelihood of remission on methotrexate (Ann Rheum Dis. 2014 Nov;73[11]:2047-53).

In the new proof-of-concept study, DMARD-naive, ACPA-positive patients with early RA undergo T-cell subset quantification by flow cytometry. Thirty patients with a normal test result are assigned to methotrexate with treat-to-target dose escalation. Based upon the investigators’ earlier work, it’s anticipated that about 80% of these patients will be in remission at 6 months.

Sixty patients with an abnormal baseline T-cell test result are being randomized to methotrexate plus either placebo or biosimilar etanercept. Again based upon the earlier study, the expected remission rate at 6 months in the methotrexate-plus-placebo group is about 20%. In contrast, the anticipated remission rate in the patients on an anti-TNF biologic plus methotrexate as first-line therapy is about 70% on the basis of the results of previous clinical trials, including PRIZE (N Engl J Med. 2014 Nov 6;371[19]:1781-92) and COMET (Ann Rheum Dis. 2012 Jun;71[6]:989-92).

Meanwhile, the price tag for biosimilar TNF inhibitors in the United Kingdom has come down to the point that routine across-the-board use of biologics as first-line therapy is arguably cost effective, a situation Dr. Emery described as hitherto “the unthinkable.”



The cost of biosimilar adalimumab in the coming year will be less than $3,000 annually in the U.K. health care system. So if 100 patients with early RA and no contraindication to biologic therapy are placed on biosimilar adalimumab and methotrexate for 1 year, the total cost for the biologic in this cohort will be less than $300,000, and roughly 70 of the 100 patients will have achieved remission. This approach makes much more sense than current standard practice, which is to reserve biologics as second-line therapy for patients who have failed to achieve remission on nonbiologic DMARDs, thereby allowing their joint damage to advance in the interim to the point that they need to stay on biologic therapy for decades, the rheumatologist argued.

“Once one accepts that remission has become the aim of therapy, then the facts, I think, speak for themselves: There’s absolutely no doubt that the rate of remission is best with the first DMARD. So if our aim is remission, we should use that one opportunity with the best agent first, because we’re not going to get the same response later,” Dr. Emery said.

Also, “there’s no doubt” that the dose of biologics can be halved with no loss of efficacy in patients who achieve remission on full-dose therapy, as previously demonstrated in PRIZE and other trials. This strategy further reduces the overall cost of biologic therapy, he added.

Dr. Emery, who recently received the Order of the British Empire from Queen Elizabeth personally in recognition of his career achievements in rheumatology, reported having no financial conflicts of interest regarding his presentation.

 

– An ongoing proof-of-concept study in the United Kingdom uses T-cell subset analysis to identify those patients with early rheumatoid arthritis who are unlikely to experience remission with methotrexate alone and therefore warrant more potent first-line therapy with a tumor necrosis factor (TNF) inhibitor in combination with methotrexate, Paul Emery, MD, said at the 2019 Rheumatology Winter Clinical Symposium.

Dr. Paul Emery, University of Leeds, England
Bruce Jancin/MDedge News
Dr. Paul Emery

“When we get the readout from this study, if we get the results we expect, we could actually put it to the National Health Service that it would be cost saving to use biologics as first-line therapy in a proportion, which will be about 40% of patients. But we won’t necessarily need to use them for a year, and we’ll get a 70%-plus remission rate, which I think is the sort of level we should be asking for in our patients,” according to Dr. Emery, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

This proof-of-concept study capitalizes on earlier work by Dr. Emery and coinvestigators, who showed in 70 patients with early rheumatoid arthritis given methotrexate as their first-ever disease-modifying antirheumatic drug (DMARD) that those with a normal pretreatment frequency of naive CD4+ T cells had an 83% remission rate at 6 months as defined by a Disease Activity Score in 28 joints (DAS28) of less than 2.6. In contrast, only 21% of those with a reduced naive CD4+ T-cell frequency experienced remission when compared with healthy controls. In an analysis adjusted for age and the presence of anti–citrullinated protein antibodies (ACPA), a normal baseline naive CD4+ T-cell frequency was associated with a 5.9-fold increased likelihood of remission on methotrexate (Ann Rheum Dis. 2014 Nov;73[11]:2047-53).

In the new proof-of-concept study, DMARD-naive, ACPA-positive patients with early RA undergo T-cell subset quantification by flow cytometry. Thirty patients with a normal test result are assigned to methotrexate with treat-to-target dose escalation. Based upon the investigators’ earlier work, it’s anticipated that about 80% of these patients will be in remission at 6 months.

Sixty patients with an abnormal baseline T-cell test result are being randomized to methotrexate plus either placebo or biosimilar etanercept. Again based upon the earlier study, the expected remission rate at 6 months in the methotrexate-plus-placebo group is about 20%. In contrast, the anticipated remission rate in the patients on an anti-TNF biologic plus methotrexate as first-line therapy is about 70% on the basis of the results of previous clinical trials, including PRIZE (N Engl J Med. 2014 Nov 6;371[19]:1781-92) and COMET (Ann Rheum Dis. 2012 Jun;71[6]:989-92).

Meanwhile, the price tag for biosimilar TNF inhibitors in the United Kingdom has come down to the point that routine across-the-board use of biologics as first-line therapy is arguably cost effective, a situation Dr. Emery described as hitherto “the unthinkable.”



The cost of biosimilar adalimumab in the coming year will be less than $3,000 annually in the U.K. health care system. So if 100 patients with early RA and no contraindication to biologic therapy are placed on biosimilar adalimumab and methotrexate for 1 year, the total cost for the biologic in this cohort will be less than $300,000, and roughly 70 of the 100 patients will have achieved remission. This approach makes much more sense than current standard practice, which is to reserve biologics as second-line therapy for patients who have failed to achieve remission on nonbiologic DMARDs, thereby allowing their joint damage to advance in the interim to the point that they need to stay on biologic therapy for decades, the rheumatologist argued.

“Once one accepts that remission has become the aim of therapy, then the facts, I think, speak for themselves: There’s absolutely no doubt that the rate of remission is best with the first DMARD. So if our aim is remission, we should use that one opportunity with the best agent first, because we’re not going to get the same response later,” Dr. Emery said.

Also, “there’s no doubt” that the dose of biologics can be halved with no loss of efficacy in patients who achieve remission on full-dose therapy, as previously demonstrated in PRIZE and other trials. This strategy further reduces the overall cost of biologic therapy, he added.

Dr. Emery, who recently received the Order of the British Empire from Queen Elizabeth personally in recognition of his career achievements in rheumatology, reported having no financial conflicts of interest regarding his presentation.

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Rheumatologist involvement often reclassifies interstitial lung disease

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Mon, 03/04/2019 - 14:06

 

– The latest practice guidelines on the diagnosis of interstitial lung disease issued by the American Thoracic Society and allied organizations recommend as the standard of care a review of all cases by a multidisciplinary team consisting of a pulmonologist, radiologist, and pathologist to ensure accurate diagnosis and classification.

Dr. Aryeh Fischer of the University of Colorado, Denver
Bruce Jancin/MDedge News
Dr. Aryeh Fischer

That’s not good enough. A rheumatologist needs to routinely be involved in those multidisciplinary discussions as well, Aryeh Fischer, MD, asserted at the 2019 Rheumatology Winter Clinical Symposium.

Why? Because a rheumatologist’s input often leads to a change in diagnosis. And that change can have important prognostic and therapeutic implications.

“We want to distinguish the IPF [idiopathic pulmonary fibrosis] patients from everybody else. The most important thing with regards to therapy is to identify the IPF patient. The IPF patients are the only ones who are able to be treated with antifibrotic agents: pirfenidone or nintedanib. And we know that immunosuppression can make patients with IPF worse; their risks of hospitalization and mortality are higher on immunosuppression. But everybody else, including anyone with any of the autoimmune diseases along with ILD [interstitial lung disease] or any other causes of ILD, gets treated with immunosuppression,” explained Dr. Fischer, a rheumatologist with a special interest in autoimmune lung disease at the University of Colorado, Denver.

He cited a recent prospective blinded study in which 60 newly diagnosed ILD patients were evaluated separately by a multidisciplinary team comprising a pulmonologist, radiologist, and pathologist and once again with the involvement of a rheumatologist. The rheumatologic assessment reclassified 21% of patients from IPF – that is, lung disease unrelated to connective tissue disease (CTD) or exposure to asbestos, bird droppings, or other triggers – to ILD with connective tissue disease (CTD-ILD). And the number of patients classified as having ILD with autoimmune features without meeting full diagnostic criteria for a major CTD, a category that includes antisynthetase syndrome and IgG4-related ILD, jumped by 77%. Also, the investigators determined that adding a rheumatologist to the multidisciplinary team would have resulted in seven fewer bronchoscopies and one less surgical biopsy among this 60-patient cohort (J Rheumatol. 2018 Nov;45[11]:1509-14).

It’s not at all uncommon to identify a new occult CTD in patients presenting with ILD. Dr. Fischer noted that in a series of 114 consecutive patients evaluated at the interdisciplinary ILD program at Johns Hopkins University, Baltimore, 30% of them had a well-defined CTD, half of whom, or 15% of the total, were newly diagnosed with CTD by virtue of their ILD evaluation (Respir Med. 2009 Aug;103[8]:1152-8).

A CTD-ILD diagnosis has prognostic implications: Survival is significantly better than with IPF, with the exception of ILD with rheumatoid arthritis (RA-ILD), where the prognosis seems to be worse than for other forms of CTD-ILD and is more akin to that of IPF. Indeed, the risk of death is threefold higher in patients with RA-ILD than in those with RA without ILD. While the predominant pattern of lung injury in RA-ILD is usual interstitial pneumonia, marked by fibrosis and honeycombing, the predominant pattern in all other forms of CTD-ILD is nonspecific interstitial pneumonia.

A French study of 778 consecutive patients with ILD highlighted how common autoimmune disease is in the ILD population. Nearly one-third of the ILD patients had autoimmune disease: 22% had CTD-ILD and another 7% had interstitial pneumonia with autoimmune features, or IPAF (Respir Med. 2017 Feb;123:56-62).

Dr. Fischer was lead author of a report by a European Respiratory Society/American Thoracic Society task force that first put forth the term IPAF to describe ILD patients with subtle serologic, clinical, and/or morphologic findings that don’t rise to the level required for formal diagnosis of a defined CTD (Eur Respir J. 2015 Oct;46[4]:976-87).

IPAF is a research construct. Patients who fall into this category are the focus of ongoing prospective studies aimed at better understanding their prognosis and appropriate treatment.

The big three autoimmune diseases comorbid with ILD as reported by Dr. Fischer and coinvestigators in a study of 237 patients with an autoimmune phenotype in a Denver ILD clinic were scleroderma, present in 37%; rheumatoid arthritis, present in 18%; and myositis, with a prevalence of 11%. Another 24% had IPAF.

“There was surprisingly little SLE [3%], and not much Sjögren’s [6%],” he observed.

When pulmonologists come knocking on Dr. Fischer’s door asking if their patients with ILD have occult CTD, he finds it helpful to look for quantifiable specific extrathoracic features suggestive of rheumatologic disease, such as Raynaud’s, sclerodactyly, mechanic hands, and Gottron’s papules.



The ILD practice guidelines recently issued jointly by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society recommend that serologic evaluation for a long list of autoantibodies “should be performed even in the absence of signs or symptoms of connective tissue disease” (Am J Respir Crit Care Med. 2018 Sep 1;198[5]:e44-e68). Dr. Fischer indicated he has a problem with that recommendation since nonrheumatologists aren’t typically adept in interpreting the significance of autoantibodies.

“I will just tell you, I see a lot of patients for ILD evaluation, and autoimmune serologies often lead to more questions than answers. They always need to be considered within the clinical context,” the rheumatologist said.

High-resolution CT (HRCT) is the imaging method of choice for detecting ILD and classifying its severity. HRCT also holds clues for detection of CTD-ILD.

“There are no upper lung–predominant ILDs that really make you think CTD. Our diseases like the lower lung zones. We like to see multicompartment involvement. When you hear ‘airways and parenchymal,’ ‘pleural,’ ‘pericardial thickening,’ that sounds a lot like autoimmune disease,” according to Dr. Fischer.

Also, the presence of a dilated esophagus on HRCT is strongly suggestive of scleroderma, he added.

In the event a pathology report is available, it’s important to read the fine print. Secondary histopathologic features of CTD-ILD include extensive pleuritis, lymphoid aggregates with germinal center formation, dense perivascular collagen, and/or prominent plasmacytic infiltration.

While ILD can be the first manifestation of an underlying occult CTD, it’s also common for a patient with an established CTD to subsequently develop ILD.

Rheumatologists are no strangers to ambiguity, so it should come as no surprise that while audible bibasilar crackles on physical examination are strongly predictive of ILD, their absence doesn’t indicate absence of ILD. Similarly, dyspnea in a patient with established CTD can be tough to interpret, and absence of dyspnea doesn’t imply absence of ILD. If a patient with CTD is on immunosuppressive therapy, bronchoalveolar lavage is of value in ruling out infection.

 

 

When to order a surgical lung biopsy

“If you’re pretty sure your patient had a CTD and then you get a characteristic HRCT and there are no exposures to account for the imaging findings – if we have a scenario where it all fits – we often don’t biopsy. Biopsy usually means a 2-night hospital stay, and it’s reportedly associated with about a 1% mortality risk. And the clinical reality is the biopsy may not impact treatment. They’re going to give you azathioprine, mycophenolate, cyclophosphamide, or prednisone for the ILD and the extrathoracic disease irrespective of the ILD pattern,” the rheumatologist said.

He reserves surgical biopsy for patients with an atypical HRCT, those with known CTD and a possible alternative etiology for the ILD, such as exposure to asbestos or owning pet birds, and patients where he’s just not sure a CTD is actually present.
 

Treatment of CTD-ILD

“The available controlled efficacy data are limited to scleroderma-ILD, where cyclophosphamide and mycophenolate work a little bit. And that’s it. We don’t have good data to guide us on which agent for which CTD or ILD pattern, for how long, or what dose,” Dr. Fischer said. “We have good drugs for the joints, nothing for the lungs. Treatment is not evidence based. We initiate with high-dose steroids, switch to a steroid-sparing agent, we evaluate the response to treatment with surveillance every 3-6 months by 6-minute walking, lung function tests, and sometimes imaging, and we treat for a long time. Oftentimes stability equals success.”

That being said, it must be emphasized that not all CTD-ILD warrants treatment of the pulmonary disease. If the patient’s ILD is mild and indolent, clinical surveillance is often appropriate, he continued.

More important than the pharmacotherapy available at present are adjunctive nonpharmacologic approaches: supplemental oxygen, pulmonary rehabilitation, treatment of comorbid GERD, immunizations, and addressing mental health issues related to these devastating diseases.

“We really don’t do these things well,” Dr. Fischer said.
 

On the horizon

An investigator-driven phase 2 clinical trial of the antifibrotic drug pirfenidone (Esbriet), now approved for IPF, is underway in patients with RA-ILD. Results of a trial of antifibrotic therapy in patients with scleroderma-ILD are due to be presented this year at EULAR. And a small study of antifibrotic therapy in patients with myositis-ILD is ongoing.

As for the biologics, there is a signal in the literature that tumor necrosis factor inhibitors may be associated with increased risk of rapidly progressive lung disease in patients with RA-ILD. An influential report from the British Society for Rheumatology Biologics Register on 299 patients with preexisting RA-ILD treated with anti-TNF therapy and 68 who received traditional disease-modifying antirrheumatic drugs showed that while mortality during follow-up was similar in the two groups, the proportion of deaths attributed to RA-ILD was 21% in the group on anti-TNF therapy, compared with only 7% in those on traditional DMARDs (Ann Rheum Dis. 2010 Jun;69[6]:1086-91).

Paul Emery, MD, rose from the audience at Dr. Fischer’s request to share his extensive experience with anti-TNF therapy and rituximab (Rituxan) in the setting of RA-ILD. When he was involved in several of the pivotal clinical trials of anti-TNF agents for RA he encountered a couple of cases of rapidly progressive ILD in patients on treatment.

“We had never before seen rapidly progressive ILD that didn’t respond to cyclophosphamide. Both patients died,” recalled Dr. Emery, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

“Our experience is if you’ve got mild ILD – and if you look hard enough you can find it in many rheumatoids – TNF inhibitor therapy doesn’t affect it. But if there’s any hint of deterioration we move away from anti-TNF therapy. Our preference has been for rituximab,” he said.

Dr. Emery was senior author of the largest study to date of rituximab in patients with RA-ILD (Rheumatology [Oxford]. 2017 Aug 1;56[8]:1348-57). While he and his coinvestigators concluded that rituximab “appears to be an acceptable therapeutic choice for patients with RA-ILD,” Dr. Fischer didn’t find persuasive evidence from this relatively small retrospective observational study in which only 44 patients had lung data available.

“My own conclusion is evidence is lacking to support a role of rituximab for treating ILD in RA,” Dr. Fischer said.

He reported receiving research grants from Boehringer-Ingelheim and Corbus and serving as a consultant to a handful of other pharmaceutical companies.

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– The latest practice guidelines on the diagnosis of interstitial lung disease issued by the American Thoracic Society and allied organizations recommend as the standard of care a review of all cases by a multidisciplinary team consisting of a pulmonologist, radiologist, and pathologist to ensure accurate diagnosis and classification.

Dr. Aryeh Fischer of the University of Colorado, Denver
Bruce Jancin/MDedge News
Dr. Aryeh Fischer

That’s not good enough. A rheumatologist needs to routinely be involved in those multidisciplinary discussions as well, Aryeh Fischer, MD, asserted at the 2019 Rheumatology Winter Clinical Symposium.

Why? Because a rheumatologist’s input often leads to a change in diagnosis. And that change can have important prognostic and therapeutic implications.

“We want to distinguish the IPF [idiopathic pulmonary fibrosis] patients from everybody else. The most important thing with regards to therapy is to identify the IPF patient. The IPF patients are the only ones who are able to be treated with antifibrotic agents: pirfenidone or nintedanib. And we know that immunosuppression can make patients with IPF worse; their risks of hospitalization and mortality are higher on immunosuppression. But everybody else, including anyone with any of the autoimmune diseases along with ILD [interstitial lung disease] or any other causes of ILD, gets treated with immunosuppression,” explained Dr. Fischer, a rheumatologist with a special interest in autoimmune lung disease at the University of Colorado, Denver.

He cited a recent prospective blinded study in which 60 newly diagnosed ILD patients were evaluated separately by a multidisciplinary team comprising a pulmonologist, radiologist, and pathologist and once again with the involvement of a rheumatologist. The rheumatologic assessment reclassified 21% of patients from IPF – that is, lung disease unrelated to connective tissue disease (CTD) or exposure to asbestos, bird droppings, or other triggers – to ILD with connective tissue disease (CTD-ILD). And the number of patients classified as having ILD with autoimmune features without meeting full diagnostic criteria for a major CTD, a category that includes antisynthetase syndrome and IgG4-related ILD, jumped by 77%. Also, the investigators determined that adding a rheumatologist to the multidisciplinary team would have resulted in seven fewer bronchoscopies and one less surgical biopsy among this 60-patient cohort (J Rheumatol. 2018 Nov;45[11]:1509-14).

It’s not at all uncommon to identify a new occult CTD in patients presenting with ILD. Dr. Fischer noted that in a series of 114 consecutive patients evaluated at the interdisciplinary ILD program at Johns Hopkins University, Baltimore, 30% of them had a well-defined CTD, half of whom, or 15% of the total, were newly diagnosed with CTD by virtue of their ILD evaluation (Respir Med. 2009 Aug;103[8]:1152-8).

A CTD-ILD diagnosis has prognostic implications: Survival is significantly better than with IPF, with the exception of ILD with rheumatoid arthritis (RA-ILD), where the prognosis seems to be worse than for other forms of CTD-ILD and is more akin to that of IPF. Indeed, the risk of death is threefold higher in patients with RA-ILD than in those with RA without ILD. While the predominant pattern of lung injury in RA-ILD is usual interstitial pneumonia, marked by fibrosis and honeycombing, the predominant pattern in all other forms of CTD-ILD is nonspecific interstitial pneumonia.

A French study of 778 consecutive patients with ILD highlighted how common autoimmune disease is in the ILD population. Nearly one-third of the ILD patients had autoimmune disease: 22% had CTD-ILD and another 7% had interstitial pneumonia with autoimmune features, or IPAF (Respir Med. 2017 Feb;123:56-62).

Dr. Fischer was lead author of a report by a European Respiratory Society/American Thoracic Society task force that first put forth the term IPAF to describe ILD patients with subtle serologic, clinical, and/or morphologic findings that don’t rise to the level required for formal diagnosis of a defined CTD (Eur Respir J. 2015 Oct;46[4]:976-87).

IPAF is a research construct. Patients who fall into this category are the focus of ongoing prospective studies aimed at better understanding their prognosis and appropriate treatment.

The big three autoimmune diseases comorbid with ILD as reported by Dr. Fischer and coinvestigators in a study of 237 patients with an autoimmune phenotype in a Denver ILD clinic were scleroderma, present in 37%; rheumatoid arthritis, present in 18%; and myositis, with a prevalence of 11%. Another 24% had IPAF.

“There was surprisingly little SLE [3%], and not much Sjögren’s [6%],” he observed.

When pulmonologists come knocking on Dr. Fischer’s door asking if their patients with ILD have occult CTD, he finds it helpful to look for quantifiable specific extrathoracic features suggestive of rheumatologic disease, such as Raynaud’s, sclerodactyly, mechanic hands, and Gottron’s papules.



The ILD practice guidelines recently issued jointly by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society recommend that serologic evaluation for a long list of autoantibodies “should be performed even in the absence of signs or symptoms of connective tissue disease” (Am J Respir Crit Care Med. 2018 Sep 1;198[5]:e44-e68). Dr. Fischer indicated he has a problem with that recommendation since nonrheumatologists aren’t typically adept in interpreting the significance of autoantibodies.

“I will just tell you, I see a lot of patients for ILD evaluation, and autoimmune serologies often lead to more questions than answers. They always need to be considered within the clinical context,” the rheumatologist said.

High-resolution CT (HRCT) is the imaging method of choice for detecting ILD and classifying its severity. HRCT also holds clues for detection of CTD-ILD.

“There are no upper lung–predominant ILDs that really make you think CTD. Our diseases like the lower lung zones. We like to see multicompartment involvement. When you hear ‘airways and parenchymal,’ ‘pleural,’ ‘pericardial thickening,’ that sounds a lot like autoimmune disease,” according to Dr. Fischer.

Also, the presence of a dilated esophagus on HRCT is strongly suggestive of scleroderma, he added.

In the event a pathology report is available, it’s important to read the fine print. Secondary histopathologic features of CTD-ILD include extensive pleuritis, lymphoid aggregates with germinal center formation, dense perivascular collagen, and/or prominent plasmacytic infiltration.

While ILD can be the first manifestation of an underlying occult CTD, it’s also common for a patient with an established CTD to subsequently develop ILD.

Rheumatologists are no strangers to ambiguity, so it should come as no surprise that while audible bibasilar crackles on physical examination are strongly predictive of ILD, their absence doesn’t indicate absence of ILD. Similarly, dyspnea in a patient with established CTD can be tough to interpret, and absence of dyspnea doesn’t imply absence of ILD. If a patient with CTD is on immunosuppressive therapy, bronchoalveolar lavage is of value in ruling out infection.

 

 

When to order a surgical lung biopsy

“If you’re pretty sure your patient had a CTD and then you get a characteristic HRCT and there are no exposures to account for the imaging findings – if we have a scenario where it all fits – we often don’t biopsy. Biopsy usually means a 2-night hospital stay, and it’s reportedly associated with about a 1% mortality risk. And the clinical reality is the biopsy may not impact treatment. They’re going to give you azathioprine, mycophenolate, cyclophosphamide, or prednisone for the ILD and the extrathoracic disease irrespective of the ILD pattern,” the rheumatologist said.

He reserves surgical biopsy for patients with an atypical HRCT, those with known CTD and a possible alternative etiology for the ILD, such as exposure to asbestos or owning pet birds, and patients where he’s just not sure a CTD is actually present.
 

Treatment of CTD-ILD

“The available controlled efficacy data are limited to scleroderma-ILD, where cyclophosphamide and mycophenolate work a little bit. And that’s it. We don’t have good data to guide us on which agent for which CTD or ILD pattern, for how long, or what dose,” Dr. Fischer said. “We have good drugs for the joints, nothing for the lungs. Treatment is not evidence based. We initiate with high-dose steroids, switch to a steroid-sparing agent, we evaluate the response to treatment with surveillance every 3-6 months by 6-minute walking, lung function tests, and sometimes imaging, and we treat for a long time. Oftentimes stability equals success.”

That being said, it must be emphasized that not all CTD-ILD warrants treatment of the pulmonary disease. If the patient’s ILD is mild and indolent, clinical surveillance is often appropriate, he continued.

More important than the pharmacotherapy available at present are adjunctive nonpharmacologic approaches: supplemental oxygen, pulmonary rehabilitation, treatment of comorbid GERD, immunizations, and addressing mental health issues related to these devastating diseases.

“We really don’t do these things well,” Dr. Fischer said.
 

On the horizon

An investigator-driven phase 2 clinical trial of the antifibrotic drug pirfenidone (Esbriet), now approved for IPF, is underway in patients with RA-ILD. Results of a trial of antifibrotic therapy in patients with scleroderma-ILD are due to be presented this year at EULAR. And a small study of antifibrotic therapy in patients with myositis-ILD is ongoing.

As for the biologics, there is a signal in the literature that tumor necrosis factor inhibitors may be associated with increased risk of rapidly progressive lung disease in patients with RA-ILD. An influential report from the British Society for Rheumatology Biologics Register on 299 patients with preexisting RA-ILD treated with anti-TNF therapy and 68 who received traditional disease-modifying antirrheumatic drugs showed that while mortality during follow-up was similar in the two groups, the proportion of deaths attributed to RA-ILD was 21% in the group on anti-TNF therapy, compared with only 7% in those on traditional DMARDs (Ann Rheum Dis. 2010 Jun;69[6]:1086-91).

Paul Emery, MD, rose from the audience at Dr. Fischer’s request to share his extensive experience with anti-TNF therapy and rituximab (Rituxan) in the setting of RA-ILD. When he was involved in several of the pivotal clinical trials of anti-TNF agents for RA he encountered a couple of cases of rapidly progressive ILD in patients on treatment.

“We had never before seen rapidly progressive ILD that didn’t respond to cyclophosphamide. Both patients died,” recalled Dr. Emery, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

“Our experience is if you’ve got mild ILD – and if you look hard enough you can find it in many rheumatoids – TNF inhibitor therapy doesn’t affect it. But if there’s any hint of deterioration we move away from anti-TNF therapy. Our preference has been for rituximab,” he said.

Dr. Emery was senior author of the largest study to date of rituximab in patients with RA-ILD (Rheumatology [Oxford]. 2017 Aug 1;56[8]:1348-57). While he and his coinvestigators concluded that rituximab “appears to be an acceptable therapeutic choice for patients with RA-ILD,” Dr. Fischer didn’t find persuasive evidence from this relatively small retrospective observational study in which only 44 patients had lung data available.

“My own conclusion is evidence is lacking to support a role of rituximab for treating ILD in RA,” Dr. Fischer said.

He reported receiving research grants from Boehringer-Ingelheim and Corbus and serving as a consultant to a handful of other pharmaceutical companies.

 

– The latest practice guidelines on the diagnosis of interstitial lung disease issued by the American Thoracic Society and allied organizations recommend as the standard of care a review of all cases by a multidisciplinary team consisting of a pulmonologist, radiologist, and pathologist to ensure accurate diagnosis and classification.

Dr. Aryeh Fischer of the University of Colorado, Denver
Bruce Jancin/MDedge News
Dr. Aryeh Fischer

That’s not good enough. A rheumatologist needs to routinely be involved in those multidisciplinary discussions as well, Aryeh Fischer, MD, asserted at the 2019 Rheumatology Winter Clinical Symposium.

Why? Because a rheumatologist’s input often leads to a change in diagnosis. And that change can have important prognostic and therapeutic implications.

“We want to distinguish the IPF [idiopathic pulmonary fibrosis] patients from everybody else. The most important thing with regards to therapy is to identify the IPF patient. The IPF patients are the only ones who are able to be treated with antifibrotic agents: pirfenidone or nintedanib. And we know that immunosuppression can make patients with IPF worse; their risks of hospitalization and mortality are higher on immunosuppression. But everybody else, including anyone with any of the autoimmune diseases along with ILD [interstitial lung disease] or any other causes of ILD, gets treated with immunosuppression,” explained Dr. Fischer, a rheumatologist with a special interest in autoimmune lung disease at the University of Colorado, Denver.

He cited a recent prospective blinded study in which 60 newly diagnosed ILD patients were evaluated separately by a multidisciplinary team comprising a pulmonologist, radiologist, and pathologist and once again with the involvement of a rheumatologist. The rheumatologic assessment reclassified 21% of patients from IPF – that is, lung disease unrelated to connective tissue disease (CTD) or exposure to asbestos, bird droppings, or other triggers – to ILD with connective tissue disease (CTD-ILD). And the number of patients classified as having ILD with autoimmune features without meeting full diagnostic criteria for a major CTD, a category that includes antisynthetase syndrome and IgG4-related ILD, jumped by 77%. Also, the investigators determined that adding a rheumatologist to the multidisciplinary team would have resulted in seven fewer bronchoscopies and one less surgical biopsy among this 60-patient cohort (J Rheumatol. 2018 Nov;45[11]:1509-14).

It’s not at all uncommon to identify a new occult CTD in patients presenting with ILD. Dr. Fischer noted that in a series of 114 consecutive patients evaluated at the interdisciplinary ILD program at Johns Hopkins University, Baltimore, 30% of them had a well-defined CTD, half of whom, or 15% of the total, were newly diagnosed with CTD by virtue of their ILD evaluation (Respir Med. 2009 Aug;103[8]:1152-8).

A CTD-ILD diagnosis has prognostic implications: Survival is significantly better than with IPF, with the exception of ILD with rheumatoid arthritis (RA-ILD), where the prognosis seems to be worse than for other forms of CTD-ILD and is more akin to that of IPF. Indeed, the risk of death is threefold higher in patients with RA-ILD than in those with RA without ILD. While the predominant pattern of lung injury in RA-ILD is usual interstitial pneumonia, marked by fibrosis and honeycombing, the predominant pattern in all other forms of CTD-ILD is nonspecific interstitial pneumonia.

A French study of 778 consecutive patients with ILD highlighted how common autoimmune disease is in the ILD population. Nearly one-third of the ILD patients had autoimmune disease: 22% had CTD-ILD and another 7% had interstitial pneumonia with autoimmune features, or IPAF (Respir Med. 2017 Feb;123:56-62).

Dr. Fischer was lead author of a report by a European Respiratory Society/American Thoracic Society task force that first put forth the term IPAF to describe ILD patients with subtle serologic, clinical, and/or morphologic findings that don’t rise to the level required for formal diagnosis of a defined CTD (Eur Respir J. 2015 Oct;46[4]:976-87).

IPAF is a research construct. Patients who fall into this category are the focus of ongoing prospective studies aimed at better understanding their prognosis and appropriate treatment.

The big three autoimmune diseases comorbid with ILD as reported by Dr. Fischer and coinvestigators in a study of 237 patients with an autoimmune phenotype in a Denver ILD clinic were scleroderma, present in 37%; rheumatoid arthritis, present in 18%; and myositis, with a prevalence of 11%. Another 24% had IPAF.

“There was surprisingly little SLE [3%], and not much Sjögren’s [6%],” he observed.

When pulmonologists come knocking on Dr. Fischer’s door asking if their patients with ILD have occult CTD, he finds it helpful to look for quantifiable specific extrathoracic features suggestive of rheumatologic disease, such as Raynaud’s, sclerodactyly, mechanic hands, and Gottron’s papules.



The ILD practice guidelines recently issued jointly by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society recommend that serologic evaluation for a long list of autoantibodies “should be performed even in the absence of signs or symptoms of connective tissue disease” (Am J Respir Crit Care Med. 2018 Sep 1;198[5]:e44-e68). Dr. Fischer indicated he has a problem with that recommendation since nonrheumatologists aren’t typically adept in interpreting the significance of autoantibodies.

“I will just tell you, I see a lot of patients for ILD evaluation, and autoimmune serologies often lead to more questions than answers. They always need to be considered within the clinical context,” the rheumatologist said.

High-resolution CT (HRCT) is the imaging method of choice for detecting ILD and classifying its severity. HRCT also holds clues for detection of CTD-ILD.

“There are no upper lung–predominant ILDs that really make you think CTD. Our diseases like the lower lung zones. We like to see multicompartment involvement. When you hear ‘airways and parenchymal,’ ‘pleural,’ ‘pericardial thickening,’ that sounds a lot like autoimmune disease,” according to Dr. Fischer.

Also, the presence of a dilated esophagus on HRCT is strongly suggestive of scleroderma, he added.

In the event a pathology report is available, it’s important to read the fine print. Secondary histopathologic features of CTD-ILD include extensive pleuritis, lymphoid aggregates with germinal center formation, dense perivascular collagen, and/or prominent plasmacytic infiltration.

While ILD can be the first manifestation of an underlying occult CTD, it’s also common for a patient with an established CTD to subsequently develop ILD.

Rheumatologists are no strangers to ambiguity, so it should come as no surprise that while audible bibasilar crackles on physical examination are strongly predictive of ILD, their absence doesn’t indicate absence of ILD. Similarly, dyspnea in a patient with established CTD can be tough to interpret, and absence of dyspnea doesn’t imply absence of ILD. If a patient with CTD is on immunosuppressive therapy, bronchoalveolar lavage is of value in ruling out infection.

 

 

When to order a surgical lung biopsy

“If you’re pretty sure your patient had a CTD and then you get a characteristic HRCT and there are no exposures to account for the imaging findings – if we have a scenario where it all fits – we often don’t biopsy. Biopsy usually means a 2-night hospital stay, and it’s reportedly associated with about a 1% mortality risk. And the clinical reality is the biopsy may not impact treatment. They’re going to give you azathioprine, mycophenolate, cyclophosphamide, or prednisone for the ILD and the extrathoracic disease irrespective of the ILD pattern,” the rheumatologist said.

He reserves surgical biopsy for patients with an atypical HRCT, those with known CTD and a possible alternative etiology for the ILD, such as exposure to asbestos or owning pet birds, and patients where he’s just not sure a CTD is actually present.
 

Treatment of CTD-ILD

“The available controlled efficacy data are limited to scleroderma-ILD, where cyclophosphamide and mycophenolate work a little bit. And that’s it. We don’t have good data to guide us on which agent for which CTD or ILD pattern, for how long, or what dose,” Dr. Fischer said. “We have good drugs for the joints, nothing for the lungs. Treatment is not evidence based. We initiate with high-dose steroids, switch to a steroid-sparing agent, we evaluate the response to treatment with surveillance every 3-6 months by 6-minute walking, lung function tests, and sometimes imaging, and we treat for a long time. Oftentimes stability equals success.”

That being said, it must be emphasized that not all CTD-ILD warrants treatment of the pulmonary disease. If the patient’s ILD is mild and indolent, clinical surveillance is often appropriate, he continued.

More important than the pharmacotherapy available at present are adjunctive nonpharmacologic approaches: supplemental oxygen, pulmonary rehabilitation, treatment of comorbid GERD, immunizations, and addressing mental health issues related to these devastating diseases.

“We really don’t do these things well,” Dr. Fischer said.
 

On the horizon

An investigator-driven phase 2 clinical trial of the antifibrotic drug pirfenidone (Esbriet), now approved for IPF, is underway in patients with RA-ILD. Results of a trial of antifibrotic therapy in patients with scleroderma-ILD are due to be presented this year at EULAR. And a small study of antifibrotic therapy in patients with myositis-ILD is ongoing.

As for the biologics, there is a signal in the literature that tumor necrosis factor inhibitors may be associated with increased risk of rapidly progressive lung disease in patients with RA-ILD. An influential report from the British Society for Rheumatology Biologics Register on 299 patients with preexisting RA-ILD treated with anti-TNF therapy and 68 who received traditional disease-modifying antirrheumatic drugs showed that while mortality during follow-up was similar in the two groups, the proportion of deaths attributed to RA-ILD was 21% in the group on anti-TNF therapy, compared with only 7% in those on traditional DMARDs (Ann Rheum Dis. 2010 Jun;69[6]:1086-91).

Paul Emery, MD, rose from the audience at Dr. Fischer’s request to share his extensive experience with anti-TNF therapy and rituximab (Rituxan) in the setting of RA-ILD. When he was involved in several of the pivotal clinical trials of anti-TNF agents for RA he encountered a couple of cases of rapidly progressive ILD in patients on treatment.

“We had never before seen rapidly progressive ILD that didn’t respond to cyclophosphamide. Both patients died,” recalled Dr. Emery, professor of rheumatology and director of the University of Leeds (England) Musculoskeletal Biomedical Research Center.

“Our experience is if you’ve got mild ILD – and if you look hard enough you can find it in many rheumatoids – TNF inhibitor therapy doesn’t affect it. But if there’s any hint of deterioration we move away from anti-TNF therapy. Our preference has been for rituximab,” he said.

Dr. Emery was senior author of the largest study to date of rituximab in patients with RA-ILD (Rheumatology [Oxford]. 2017 Aug 1;56[8]:1348-57). While he and his coinvestigators concluded that rituximab “appears to be an acceptable therapeutic choice for patients with RA-ILD,” Dr. Fischer didn’t find persuasive evidence from this relatively small retrospective observational study in which only 44 patients had lung data available.

“My own conclusion is evidence is lacking to support a role of rituximab for treating ILD in RA,” Dr. Fischer said.

He reported receiving research grants from Boehringer-Ingelheim and Corbus and serving as a consultant to a handful of other pharmaceutical companies.

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