Recent findings, new classification criteria and treatment guidelines, and concerted efforts by various organizations to provide educational resources are among a number of factors improving the outlook for patients with primary Sjögren’s syndrome, according to Judith James, MD, PhD.
Dr. Judith James
Additionally, the number of studies of primary Sjögren’s syndrome (pSS) is increasing, albeit slowly, and ongoing studies of biologics are showing promise, Dr. James said during a clinical update at the Winter Rheumatology Symposium sponsored by the American College of Rheumatology.
Classification criteria
The ACR in conjunction with the European League Against Rheumatism (EULAR) published new criteria for pSS classification in 2016 based on the available evidence and expert consensus. Inclusion criteria include daily, persistent, troublesome dry eyes for more than 3 months, recurrent sensation of sand or gravel in the eyes, use of tear substitutes more than three times each day, frequent drinking of liquids to aid in swallowing dry food, or at least one EULAR Sjögren’s syndrome disease activity index (ESSDAI) domain with a positive item. Exclusion criteria include prior head and neck radiation treatment, polymerase chain reaction–confirmed active hepatitis C infection, AIDS, sarcoidosis, amyloidosis, graft-versus-host disease, or IgG4-related disease, said Dr. James, professor and chair of the arthritis and clinical immunology research program at the University of Oklahoma, Oklahoma City (Arthritis Rheumatol. Jan 2017;69[1]:35-45).
- A score of 4 or higher in patients who meet the inclusion criteria and do not have any of the exclusion criteria leads to classification with pSS, based on the following findings:
- Labial salivary gland with focal lymphocytic sialadenitis and focus score of at least 1 foci/4 mm2 (weight/score = 3).
- Anti-SSA/Ro-positivity (weight/score = 3).
- Ocular Staining Score of at least 5, or van Bijsterveld score of at least 4, in at least one eye (weight/score = 1).
- Schirmer’s test of no more than 5 mm/5 min in at least one eye (weight/score = 1).
- Unstimulated whole saliva flow rate of no more than 0.1 mL/min (weight/score = 1).
Clinical pearls for detection and management
In Dr. James’ experience, the three symptoms (taken together) with the highest predictive value for diagnosing pSS are dry mouth, sore mouth/tongue, and dry eyes. About 25% of Sjögren’s patients may have no detectable salivary flow, she said.
Dry, cracked skin that can lead to secondary infections is another common issue affecting about 55% of patients.
“So we always have to talk to our Sjögren’s patients about skin,” she said. “We also have recurrent sinusitis, chronic cough, dyspepsia, constipation, and other symptoms.”
Concurrent autoimmune diseases are another concern in Sjögren’s patients, she said. One to particularly keep in mind, in addition to lupus and rheumatoid arthritis, is autoimmune thyroid disease.
Data suggest that up to 45% of Sjögren’s patients have thyroid dysfunction, and if you look at just those with autoimmune thyroiditis, their risk of Sjögren’s is increased 10-fold vs. those without autoimmune thyroiditis, she said.
Other conditions to keep in mind when it comes to diagnosing and managing patients, as has been shown in numerous studies over the years, include Raynaud’s phenomenon, which affects at least 13% of patients, and subclinical muscle inflammation, which affects more than 50% of patients, Dr. James said.
“Depression ... as well as anxiety, is quite common in Sjögren’s patients, and fatigue is profound,” she added, noting that fatigue is “the No. 1 issue” for many patients.
Another area of particular concern in Sjögren’s is the increased risk of lymphoma, she said.
Studies show varying rates of lymphoma in Sjögren’s, with one suggesting a 44-fold increased risk, but this is likely only among those at very high risk. Other studies suggest the increase is risk overall is in the range of 4- to 10-fold, she said.