Sjogren's Syndrome Foundation's 2017 Sjogren's Treatment Guidelines Save

Jack Cush, MD

Mar 23, 2017 9:00 am

Dr. Steven Carsons and an expert committee of the Sjogren's Syndrome Foundation have published a clinical practice guidelines (CPGs) and recommendations for the managment of Sjögren's syndrome.

A CPG committee (with input from patients and rheumatologists) was convened and reviewed the literature to address specific clinical questions. Guideline recommendations were devised and reviewed by a consensus expert panel (CEP) composed of 30–40 clinicians from academia and community practices. A guideline recommendation required a CEP agreement level of 75%. Overall, 19 recommendations were put forth.

These recommendations (and the strength of evidence) address when to use oral disease-modifying antirheumatic drugs or biologic agents, the use of self-care measures how to manage fatigue, and the use. Rituximab in was recommended in only selected settings - including ocular dryness, extraglandular manifestations, vasculitis, severe parotid swelling, inflammatory arthritis, pulmonary disease, and mononeuritis multiplex. The use of tumor necrosis factor inhibitors for primary Sjögren's syndrome was discouraged.

Recommendations on Use of Biologics	Strength
1. TNF inhibitors should NOT be used to treat sicca symptoms	Strong
2. If TNFi used in SS, patients should be monitored for lymphoma, malignancies, serious infections, TB, fungal infections, etc. (see long list in paper taken from package insert)	Strong
3. There is weak evidence that Rituximab may be considered as a therapeutic option for KCS in patients with primary Sjögren's syndrome	Weak
4. There is weak evidence that Rituximab may be considered as a therapeutic option for xerostomia in patients with primary Sjögren's syndrome	Weak
5. Rituximab may be considered as a therapeutic option for adults with primary Sjögren's syndrome with any or all of the following systemic manifestations: Cryoglobulinemia, vasculitis, severe parotid swelling, inflammatory arthritis, pulmonary disease, peripheral neuropathy, and mononeuritis.	Moderate
6. If using RTX, should monitor for uncommon infusion related problems (see long list in paper taken from package insert)	Strong

Recommendations for Fatigue	Strength
7.Education about self-care measures should include advice about exercise to reduce fatigue	Strong
8. DHEA is NOT recommended for treatment of fatigue in Sjögren's syndrome	Strong
9. Hydroxychloroquine may be considered in selected situations to treat fatigue in Sjögren's	Weak
10. TNF inhibitors are NOT recommended for treatment of fatigue in Sjögren's syndrome	Strong

Recommendations for Inflammatory Musculoskeletal (MSK) Pain	Strength
11.Hydroxychloroquine may be first line treatment for inflammatory MSK pain	Moderate
12. Methotrexate may be considered If HCQ is not effective in treating inflammatory MSK pain	Moderate
13. If neither MTX or HCQ is effective, both HCQ plus MTX may be considered	Moderate
14. If MTX and HCQ is not effective, short-term (≤1 month) corticosteroids of ≤15 mg/day may be considered	Strong
15.If long term steroids are being used, efforts should be made to use a steroid-sparing agent	Moderate
16. Leflunomide may be considered in treating inflammatory MSK pain	Weak
17. Sulfasalazine may be considered in treating inflammatory MSK pain	Weak
18. Azathioprine may be considered in treating inflammatory MSK pain	Moderate
19, Cyclosporine may be considered in treating inflammatory MSK pain	Weak

These recommendations suffer by being recommendations that rely on clinical trials that are hampered by design, lack of comparative controls and a lack of defined standards for "clinically meaningful improvement" with many of the agents noted above.

Another problem with these recommendations is the use of DMARDs for the management of inflammatory MSK pain. What is inflammatory MSK pain? It is not defined in the methods and doesn't appear to be the same as "inflammatory arthritis". Most patients with Sjogrens don't have polysynovitis or elevated acute phase reactants and maybe this is why many of our best rheumatoid therapies have failed in Sjogrens. Many patients (up to 60%) with Sjogrens syndrome have soft tissue and myofascial pains and fibromyalgic symptoms. The authors point out that inflammatory MSK pain ranges from mild arthralgias and myalgias to frank synovitis with chronic pain. Its hard to uniformly advocate for DMARD therapy (e.g., MTX< HCQ, LEF, SSZ, AZA, CyA) for this range of joint complaints. Moreover, there have been numerous trials showing the lack of improvement in sicca symptoms when DMARDs or biologics were studied.

These recommendations mark a significant step forward in advocating for a treatment plan with options for those Sjogrens patients who have more than sicca symptoms, including fatigue, joint symptoms and extraarticular and systemic manifestations of this common disorder.

Treatment Guidelines for Rheumatologic Manifestations of Sjögren's Syndrome: Use of Biologic Agents, Management of Fatigue, and