ORLANDO – The according to Ruth Ann Vleugels, MD, director of the autoimmune skin diseases program at Brigham and Women’s Hospital, Boston.
“We will have patients who essentially fail all of our typical therapies and are still coming to us for help. This is a huge challenge,” said Dr. Vleugels , who, several years ago, started to use tofacitinib to treat these patients. “Similar to my colleagues who use tofacitinib to treat alopecia areata, we often have to push” beyond the dose used to treat rheumatoid arthritis, to 10 mg twice a day, she said at the International Conference on Cutaneous Lupus Erythematosus. Tofacitinib helps counter the overexpression of interferon in DM.
Dr. Ruth Ann Vleugels Dr. Vleugels said she has treated 10 or so DM patients with severe refractory skin disease; they have had meaningful decreases in cutaneous disease activity scores and less itching, and they have been able to come off other therapies. For now, she keeps tofacitinib in reserve mostly for patients who cannot tolerate intravenous immunoglobulin (IVIg). She has also been involved in a pilot study of tofacitinib for refractory DM in adults, funded in part by the drug’s manufacturer, Pfizer.
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Getting insurance coverage for this off-label indication can be tough, however, but Dr. Vleugels said she’s had success when she tells insurers that tofacitinib will likely reduce the need for IVIg.
It’s safe to keep patients on methotrexate if there are concerns about muscle involvement while their skin is brought under control with tofacitinib. In terms of side effects, “we see increased shingles,” so recommending the shingles vaccine for these patients is a good idea, she added.
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Dr. Alisa Femia
It’s also important to counsel DM patients that they are at particular risk for skin reactions with antimalarials, which can be serious, so that, “if there is a drug reaction that develops, it’s noticed right away” and the drug can be stopped, she said. “If you have a patient who has very severe disease, I might skip over an antimalarial altogether,” she commented.
Methotrexate is the next option, especially if there are work ups for cancer or the patients have cancer, but Dr. Femia, director of inpatient dermatology at NYU, said she leans towards mycophenolate if there’s concern about lung involvement.
The next step, if necessary, is IVIg, which she said is “particularly helpful” for recalcitrant skin disease and can help some patients discontinue other immunosuppressives. To counter headache, a common side effect, she will space dosing out over 3 days, instead of the usual 2, and have a bag of saline administered before and after the infusion to keep patients hydrated; this counters the headache-inducing viscosity of IVIg.
Patients often see a result after the first infusion, but if there’s no benefit by the third cycle, “it’s probably time to move on,” she said. “If you have a refractory muscle disease patient and skin isn’t the main issue, rituximab is reasonable to try,” she added, noting that the benefit of tumor necrosis factor blockers, “at best, is very mixed in the DM population. They are very low down in the treatment algorithm.”
Dr. Vleugels and Dr. Femia are both Pfizer investigators.