Sjogren's Syndrome Foundation Clinical Practice Guidelines: "A Dry Topic"

The Sjogren's Syndrome Foundation developed a series of clinical practice guidelines for the important manifestations of Sjogren's Syndrome including (a) dry eyes, (b) oral disease (management of dental caries) and (c) systemic disease management. Using the method of AGREE and GRADE principles to assess the strength of recommendations, a group of experts including rheumatologists, eye and oral care providers presented a series of posters at ACR outlining these recommendations.

Key recommendations are presented below according to each manifestation:

(a) Dry eyes: Subdivided between those with and without meibomian gland disease, initial recommendations included education, removal of offending medications, artificial tears, gels and ointments. Within increasing levels of severity (four subdivisions), recommendations included omega-3 essential fatty acid supplements, pulse steroids, cyclosporine, punctual plugs, secretagogues, and contact lenses. At the greatest level of severity, eyelid surgery or systemic anti-inflammatory medications are recommended, although the strength of recommendation for anti-inflammatory medication is weak.

(b) Oral disease management: Caries Prevention: Questions related to use of fluoride, salivary stimulation, antimicrobials and non-fluoride remineralizing agents were evaluated. One strong recommendation resulted, supporting the use of topical fluoride in Sjogren’s patients with dry mouth. In contrast, a weak recommendation based on no studies suggested that Sjogren's patients should increase salivation through gustatory, masticatory stimulation, and pharmaceutical agents. Use of chlorhexidine by varnish/gel/or rinse was recommended in Sjogren's patients with dry mouth and a high rate of root caries. A moderate strength recommendation was made to support non-fluoride remineralizing agents as an adjunct in Sjogren's patients with dry mouth and a high rate of root caries.

(c) Systemic disease: Recommendations for systemic disease were subdivided into (a) biological therapies, (b) DMARDs for inflammatory MSK pain and (c) fatigue. A strong recommendation was made to NOT use TNF-inhibitors for sicca symptoms for primary Sjogren's while a moderate strength recommendation was made for rituximab for primary Sjogren's patients with cryoglobulinemic vasulitis, vasculitis, severe parotid swelling, inflammatory arthritis, pulmonary disease or peripheral neuropathy (especially mononeuritis). Rituximab was recommended (weak strength) for xerostomia in Sjogren's patients in specific scenarios (e.g., evidence of residual salivary production, oral damage, failure of other treatments). Regarding DMARDs for inflammatory MSK pain in primary Sjogren's, only one strong recommendation was made for using short-term corticosteroids (< 1 month, < 15 mg prednisone) when hydroxychloroquine and methotrexate were not effective. Other moderate strength recommendations for inflammatory MSK pain in primary Sjogren's essentially followed the treatment algorithm used in rheumatoid arthritis, with use of hydroxychloroquine, methotrexate, sulfasalazine, leflunomide, cyclosporine and azathioprine sequentially, depending on treatment response. Strong recommendations relating to treating fatigue in primary Sjogren'’s included recommending the use of exercise and NOT the use of DHEA (dehyroepiandrosterone), infliximab or etanercept. A weak recommendation was made for hydroxychloroquine in fatigue in certain clinical contexts.

Overall, these recommendations are based on limited evidence of limited quality. However, an increasing role for rituximab and, to a lesser extent DMARDs, for systemic manifestations of primary Sjogren's patients, may offer new therapeutic options for some of our patients.

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