What are the EULAR treatment guidelines for systemic disease in Sjögren syndrome?

Updated: Mar 05, 2021
  • Author: Sriya K Ranatunga, MD, MPH; Chief Editor: Herbert S Diamond, MD  more...
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Systemic disease:

  • Treatment of systemic disease should be tailored to organ-specific severity using the EULAR Sjögren's syndrome disease activity index (ESSDAI) definitions.
  • Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease (eg, pulses of methylprednisolone followed by doses of 0.5 mg/kg/d or lower as induction therapy in severe presentations, and doses< 0.5 mg/kg/d in moderate/less-severe presentations, with a final target of withdrawing glucocorticoids in patients with inactive disease as soon as possible or at least trying to target a maintenance dose of 5 mg/daily or less with the aid of glucocorticoid-sparing immunosuppressive agents).
  • Immunosuppressive agents (eg, leflunomide, methotrexate, azathioprine, mycophenolate, cyclophosphamide) should be mainly used as glucocorticoid-sparing agents, with no evidence supporting the choice of one agent over another.            
  • B-cell–targeted therapies (eg, rituximab, epratuzumab, belimumab) may be considered in patients with severe, refractory systemic disease.
  • The systemic organ-specific therapeutic approach may follow, as a general rule, the sequential (or combined) use of glucocorticoids, immunosuppressive agents, and biologics.
  • Treatment of B-cell lymphoma should be individualized according to the specific histological subtype and disease stage.

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