First Clinical Practice Guidelines for Sjögren’s Syndrome Developed

salivary gland of a person with Sjögren's syndrome
salivary gland of a person with Sjögren’s syndrome
A clinical expert panel from the Sjögren's Syndrome Foundation has published the first-ever clinical practice guidelines for the disease in the United States.

The Sjögren’s Syndrome Foundation has released the first clinical practice guidelines for the disease, which includes recommendations on the management of fatigue, inflammatory musculoskeletal pain, and use of biologic agents.

Steven E. Carsons, MD, from the Division of Rheumatology and Allergy and Immunology at Winthrop University Hospital in Mineola, New York, and colleagues said they developed the Sjögren’s Syndrome Foundation clinical practice guidelines after “patient requests for improved care and physician requests for guidance.”

The Sjögren’s Syndrome Foundation clinical expert panel consisted of clinicians, nurses, and patients who, using a modified Delphi process (75% agreement level), achieved consensus on 19 different recommendations. Three topic review groups were created on the issues of musculoskeletal pain, fatigue, and biologic use, in which panelists performed systematic reviews of the MEDLINE/PubMed and Cochrane databases and compiled data on the topics published between January 1988 and April 2015. A summary of the recommendations was recently published in Arthritis Care & Research.

“Among all chronic autoimmune rheumatic disorders, Sjögren’s syndrome remains one of the most difficult to manage,” Dr Carsons and colleagues wrote in their recommendations. “Development of [clinical practice guidelines] for the ocular, oral, and systemic/rheumatologic manifestations should substantially improve the quality and consistency of care, guide reimbursement policies, and decrease the overall burden of illness.”

Regarding biologic therapy for sicca symptoms and systemic manifestations of Sjögren’s syndrome, the panelists recommended against using tumor necrosis factor (TNF) inhibitor therapy, and in cases in which these patients are using TNF inhibitor therapy to treat rheumatoid arthritis, the panelists recommended looking for toxicities such as lymphoma, serious infections, invasive fungal infections, hepatitis B, hepatoxicity, heart failure, cytopenias, hypersensitivity, and demyelinating disease.

The panelists indicated rituximab for use in patients with primary Sjögren’s syndrome and conditions such as keratoconjunctivitis, vasculitis, severe parotid swelling, pulmonary disease, severe inflammatory arthritis with or without cryoglobulinemia, peripheral neuropathy, and xerostomia with primary Sjögren’s syndrome “with some evidence of residual salivary production and significant evidence of oral damage, as determined by the clinician, and for whom conventional therapies, including topical moisturizers and secretagogues, have proven insufficient.”

To treat fatigue in patients with Sjögren’s syndrome, the panelists recommended clinicians mention exercise as a self-care measure and noted evidence for improvement in patients with systemic lupus erythematosus, rheumatoid arthritis, and multiple sclerosis. Although evidence was low for hydroxychloroquine (HCQ) use in treating fatigue in patients with Sjögren’s syndrome, the panel recommended its use based on clinical experience and a good safety profile. However, the panels did not recommend treating fatigue in patients with Sjögren’s syndrome using dehydroepiandrosterone based on 2 randomized controlled trials showing no difference between the treatment and placebo.

“Neither of the TNF inhibitors etanercept or infliximab is recommended for treatment of fatigue in Sjögren’s syndrome,” Dr Carsons and colleagues wrote in their recommendation summary. “Newer biologic agents, in the opinion of the [topic review group], have insufficient data and/or clinical experience to make a recommendation regarding the use of anakinra, abatacept, belimumab, and epratuzumab for fatigue in Sjögren’s syndrome.”

The panelists used a decision tree to outline their recommendations for use of disease-modifying antirheumatic drugs for treating musculoskeletal pain, with HCQ as a first-line treatment, followed by methotrexate alone if HCQ is not effective. If neither treatment is effective alone, the panelists recommended combination HCQ plus methotrexate, and short-term corticosteroids (<15 mg/day) if that combination therapy was not effective. The next treatment recommendation was leflunomide, followed by sulfasalazine if that treatment proved ineffective. Panelists concluded clinicians should consider azathioprine for patients who do not respond to those treatments, and cyclosporine for patients who do not respond to any of the other treatments.

“There was strong agreement (92%) among the [clinical expert panel] that if major organ involvement occurs in the primary Sjögren’s syndrome patient, azathioprine would be a better choice than leflunomide or sulfasalazine for the treatment of all extraglandular manifestations, including inflammatory [musculoskeletal] pain,” the panelists wrote.

Summary and Clinical Applicability

“The guidelines provide evidence-based recommendations whenever possible and expert opinion when insufficient evidence exists,” Dr Carsons and colleagues wrote. “These first-ever standard-of-care guidelines for systematic Sjögren’s syndrome in the US will improve consistency in practice patterns, inform coverage and reimbursement policies, lead to the design and implementation of needed educational programs, highlight areas for future research, and, most importantly, fill a significant clinical void.”


Dr Carsons has received consulting fees from Biogen Idec and honoraria from NS-LIJ Health Systems, and serves on the advisory board for Nicox (less than $10,000 each). Dr Vivino has received consulting fees from Biogen Idec, Nicox, Immco Diagnostics, and Takeda (less than $10,000 each). Dr Parke has received consulting fees from UCB, GSK, and Biogen Laboratories (less than $10,000 each). Dr Carterton has received consulting fees from Valeant Pharmaceuticals (less than $10,000), owns stock in Genentech/Roche, receives book royalties from Penguin and from the Taylor and Francis Group, and is on the board of directors of Healthwell Foundation. Dr Brasington has received consulting fees from Pfizer (less than $10,000) and has an immediate family member who owns stock in Amgen. Dr Fox has received consulting fees from UCB, Takeda, and Pfizer (less than $10,000 each) and has received consulting fees from Allergan (more than $10,000). Dr Scofield has received consulting fees from UCB and honoraria from Eli Lilly (less than $10,000 each).

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Carsons SE, Vivino FB, Parke A, et al. Treatment guidelines for rheumatologic manifestations of Sjögren’s syndrome: use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain. Arthritis Care Res. 2017;69(4):517-527. doi:10.1002/acr.22968

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