This protocol addresses patients with Sjögren syndrome presenting with cutaneous vasculitis as a manifestation of active systemic disease. Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions. The best indication for escalation is probably for symptoms linked to cryoglobulinemic-associated vasculitis.
This protocol applies when a prior course of immunosuppressive agents as glucocorticoid-sparing therapy — including leflunomide, methotrexate, azathioprine, mycophenolate, or cyclophosphamide — has failed to achieve a reduction of ≥3 points in the global ESSDAI score.
When that target is not reached, a more targeted next-line approach is indicated.
The next step for this refractory presentation involves a targeted biologic agent, with particular relevance when cutaneous vasculitis has a cryoglobulinemic component. The complete regimen — including agent selection, dosing, and administration schedule — is available in the full protocol.
Treatment goal: Reduction of ≥3 points in the global ESSDAI score.
DOI: 10.1136/annrheumdis-2019-216114
Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
The best indication is probably for symptoms linked to cryoglobulinemic-associated vasculitis.
The use of rituximab may be considered in patients with severe, refractory systemic disease, and that the best indication is probably for symptoms linked to cryoglobulinemic-associated vasculitis.
With respect to the definition of the therapeutic response in systemic SjS, the TF recommends using a reduction of >=3 points in the global ESSDAI score.
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