The Sjogren’s Syndrome Foundation has issued the first-ever treatment guidelines for systemic Sjogren’s syndrome, a disease that affects as many as 6 million people in the U.S. with or without connective tissue disease.
Sjogren’s syndrome is one of the most difficult chronic autoimmune rheumatic disorders to treat, wrote the authors of the recommendations that appear in the March 2017 print issue of Arthritis Care & Research. “It is difficult to distinguish between disease activity and damage, and therefore it is challenging to identify active cases to achieve a meaningful response,” the authors wrote.
Because there is no-known cure, the treatment recommendations are designed to alleviate symptoms, improve quality of life, prevent further damage and help physicians select the appropriate immunosuppressive treatment for patients.
Led by Steven E. Carsons, M.D., of Winthrop University Hospital in Mineola, New York, the physician committee outlined 19 recommendations which center on the use of biologic agents, the management of fatigue and inflammatory musculoskeletal pain.
The guidelines are comprehensive, so here, we highlight some important points:
- Sicca: The committee recommends against using TNFi therapy to treat sicca symptoms in patients with primary Sjogren’s syndrome. This recommendation is based on a small controlled trial and a multicenter trial that showed little it no improvement in these patients.
- Non-Hodgkin’s B cell lymphoma: Despite theoretical concerns of increased risk of non-Hodgkin’s B cell lymphoma in Sjogren’s patients, the research team found no evidence of increased lymphoma in a subset of rheumatoid arthritis patients who also had Sjogren’s syndrome and were treated with anti-TNF agents.
Rituximab uses in Sjogren’s syndrome:
- Rituximab may be considered as a therapeutic option for keratoconjunctivitis sicca in patients with primary Sjogren’s syndrome for whom conventional therapies (topical moisturizers, secretagogues, antiinflammatory agents, immunomodulators, and punctual occlusion) have proven insufficient.
- Rituximab may be considered as a therapeutic option for xerostomia in patients with primary Sjogren’s syndrome with evidence of residual salivary production and significant evidence of oral damage for whom conventional therapies have been insufficient.
- Rituximab may be considered as a therapeutic option for adults with primary Sjogren’s syndrome and any of the following: vasculitis (with or without cryoglobulinemia), severe parotid swelling, inflammatory arthritis, pulmonary disease, and peripheral neuropathy (especially mononeuritis multiplex).
- Rituximab to treat dry eyes and/or dry mouth would only be appropriate in severe cases and with the input from the patient’s ocular and/or oral medicine specialist.
Other important findings:
- Dehydroepiandrosterone (DHEA) is not recommended for treatment of fatigue in Sjogren’s syndrome (this is based on the results of clinical trials in which patients experienced no improvements).
- TNF inhibitors etanercept and infliximab are not recommended for treatment of fatigue in Sjogren’s syndrome.
- There was insufficient data to recommend the use of newer biologic agents - such as anakinra, abatacept, belimumab, and epratuzumab - for fatigue in Sjogren’s syndrome.
- The guidelines outline recommendations for the use of DMARDs for inflammatory musculoskeletal pain as a decision tree with the first-line treatment as hydroxychloroquine. Other DMARDs in certain cases, or in more severe cases, may be warranted.
- The causes of fatigue in Sjogren’s syndrome are numerous. The only strong therapeutic recommendation for fatigue in Sjogren’s syndrome is exercise.
- Hydroxychloroquine is the most widely prescribed treatment in the U.S. to manage fatigue in Sjogren’s syndrome, but this practice is largely made based on clinical experience.
Development of the guidelines was done with support from the Sjogren’s Syndrome Foundation.
Steven E. Carsons, Frederick B. Vivino, Ann Parke, et al. “Treatment Guidelines for Rheumatologic Manifestations of Sj€ogren’s Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain,” Arthritis Care & Research. March 2017. DOI 10.1002/acr.22968