This protocol addresses patients with active systemic Sjögren syndrome who have symptomatic cryoglobulinemia with positive serum cryoglobulins. Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions, and the best indication for escalation is for symptoms linked to cryoglobulinemic-associated vasculitis.
This protocol is indicated when glucocorticoids — oral for moderate disease, or intravenous methylprednisolone pulses followed by oral for severe disease — have not achieved the required treatment target: a reduction of ≥3 points in the global ESSDAI score.
When glucocorticoid therapy has not produced an adequate ESSDAI response in the setting of symptomatic cryoglobulinemia, rituximab may be considered as the next therapeutic step. The specific dosing schedule and administration details are defined in the structured protocol.
Treatment goal: Reduction of ≥3 points in the global ESSDAI score
DOI: 10.1136/annrheumdis-2019-216114
The best indication is probably for symptoms linked to cryoglobulinemic-associated vasculitis.
Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
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.
View source ↗