Background/Purpose: There are currently no FDA approved immunomodulating agents available for treatment of the extraglandular manifestations of Sjogren’s (SS).  Clinical practice guidelines were developed for the rheumatologic/systemic manifestations of SS in order to improve quality and consistency of care.

Methods: Following the principles of AGREE and ACR recommendations for guidelines development, key stakeholders were surveyed to define clinically important management issues. Of 97 initial questions, 16 were selected for rheumatology guidelines development, redefined using the PICO (Patient, Intervention, Comparison, Outcome) format and prioritized. Guidelines development was completed for the first 3 topics: use of DMARDS for treatment of inflammatory musculoskeletal pain (MSK), management of fatigue and use of biological therapy. Each PICO question was assigned to a 3 member topic review group (TRG) for systematic literature review, data extraction and drafting of one or more guidelines. All articles were reviewed by at least 2 TRG members. Quality of evidence and strength of recommendation were rated using a modification of GRADE. Guidelines were drafted based on the available medical evidence and/or expert opinion. The clinical rationale, evidence summaries, data extraction tables, and drafted recommendations were submitted to a consensus expert panel for consideration and approval. Each panel was comprised of 30-40 participants including clinicians (rheumatologists, other specialists), nurses and patients.  A modified Delphi process was used with 75% agreement required for consensus. Revision of guidelines that failed to achieve consensus was permitted up to 3 rounds before the recommendation was discarded.

Results: Consensus was achieved for 20 guideline recommendations.  Eighteen recommendations required 1 Delphi round, 1 required 2 and 1 required 3.  Key recommendations included: 1) a decision tree for the use of oral DMARDs for MSK with hydroxychloroquine (HCQ) as first line therapy; 2) use of self-care measures and exercise to reduce fatigue and the use of HCQ in selected patients with fatigue. The committee also recommended that 3) the use of rituximab may be considered in certain clinical situations including, vasculitis with or without cryoglobulinemia, severe parotid swelling, inflammatory arthritis, pulmonary disease and mononeuritis multiplex;  The committee 4) strongly discouraged the use of TNFα inhibitors for sicca symptoms and for the majority of clinical contexts in primary SS.

Conclusion: Development of clinical practice guidelines for systemic manifestations should improve the standard and uniformity of care for SS and help define areas for future study. The guidelines committee updates its systematic reviews regularly and will revise recommendations as needed.  

The Sjögren’s Syndrome Foundation (SSF) Clinical Practice Guidelines are fully supported by the SSF with no pharmaceutical support.  No compensation was paid to any author. All participating authors completed Conflict of Interest forms of the American College of Rheumatology.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/sjogrens-syndrome-foundation-clinical-practice-guidelines-for-management-of-systemic-disease/