March 22, 2017
3 min read

Sjögren's Syndrome Foundation releases its first guidelines for disease treatment

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The Sjögren’s Syndrome Foundation has released its first treatment guidelines for the management of Sjögren’s syndrome, according to a recently published report.

“Among all chronic autoimmune rheumatic disorders, Sjögren’s syndrome remains one of the most difficult to manage,” Steven E. Carsons, MD, at Winthrop-University Hospital Campus at the State University of New York, and colleagues wrote. “Development of [clinical practice guidelines] CPGs 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.”

Carsons and colleagues formed separate topic review groups that performed systematic literature reviews and extracted data to produce their recommendations. They rated quality of evidence and strength of recommendation according to the American Society of Clinical Oncology’s modification of the Grading of Recommendations Assessment Development, and Evaluation. A consensus expert panel (CEP) of 30 to 40 clinicians from academia and community practices, as well as nurses and patients, reviewed the recommendations. A CEP agreement level of 75% led to adoption of the recommendation. Overall, the group formed 19 recommendations, the following 13 of which were considered to be strong or moderate in the management of Sjögren’s syndrome:

  • Tumor necrosis factor (TNF) inhibitors should not be used to treat sicca symptoms;
  • If TNF inhibitor therapy is being used for rheumatoid arthritis (RA) or another overlapping condition, health care providers should monitor lymphoma and other malignancies, serious infections including — tuberculosis — invasive fungal infections, hepatitis B reactivation, hepatotoxicity, heart failure, cytopenias, hypersensitivity, serious infection reactions and demyelinating disease;
  • Rituximab can be considered for adults who also have cryoglobulinemia associated with vasculitis, vasculitis, severe parotid welling, inflammatory arthritis, pulmonary disease and peripheral neuropathy, specifically mononeuritis;
  • If the patient is on rituximab, health care providers should be aware of infusion reactions, tumor lysis syndrome in those with non-Hodgkin’s lymphoma, progressive multifocal leukoencephalopathy, hepatitis B reactivation with possible fulminant hepatitis, severe mucocutaneous reactions, infections, bowel obstruction and perforation, cardiac arrhythmias and angina, cytopenias and serious bacterial viral or fungal infections. In addition, they should avoid administration of live vaccines and they should perform an assessment of risk and benefit of rituximab if the patient is pregnant or nursing;
  • Education about self-care should include use of exercise to reduce fatigue;
  • Dehydroepiandrosterone should not be used to treat fatigue;
  • Neither etanercept nor infliximab should be used to treat fatigue;
  • Hydroxychloroquine (HCQ) should be the first line of treatment for inflammatory musculoskeletal (MSK) pain;
  • If HCQ is not effective for the treatment of inflammatory MSK pain, methotrexate alone can be considered;
  • If either HCQ or methotrexate alone is not effective in the treatment of inflammatory MSK pain, combination therapy of HCQ plus methotrexate can be considered;
  • If HCQ plus methotrexate is not effective in treatment of inflammatory MSK pain, short-term corticosteroids of fewer than 15 mg per day for 1 month or less may be considered;
  • Long-term corticosteroids of at least 15 mg per day for more than 1 month can be considered in the management of inflammatory MSK pain, but efforts should be made to find a steroid-sparing agent as quickly as possible; and
  • If major organ involvement occurs, azathioprine may be a better choice than leflunomide or sulfasalazine for the treatment of all extra glandular manifestations, including inflammatory MSK pain.

“Although HCQ remains the first-line therapy for inflammatory MSK pain in Sjögren’s syndrome, clinicians may choose other [disease-modifying antirheumatic drugs] DMARDs in certain situations or in more severe cases where the perceived benefits outweigh the risk of increased toxicity,” the researchers wrote. “These clinical scenarios include HCQ-responsive patients who must discontinue this therapy due to toxicity or an adverse effect, patients with an inadequate response to HCQ, patients with severe steroid-responsive MSK pain and persistent symptoms who require another DMARD for steroid-sparing effect, and patients with objective evidence of synovitis.” – by Will Offit


Disclosures: Carsons reports consulting fees from Biogen Idec, honoraria from NS-LIJ Health Systems and serves on the advisory board for Nicox, earning less than $10,000 for each. Please see the full study for a list of all other relevant financial disclosures.