In patients with Sjögren syndrome presenting with myelitis as part of active systemic disease, first-line immunosuppressive treatment targets stabilisation of CNS involvement and reduction of overall disease activity. When that initial approach does not achieve the required response, a structured next-line protocol defines the appropriate escalation.
Sjögren syndrome with myelitis and active systemic disease — CNS manifestations including demyelinating disease and myelitis are the defining features of this presentation.
First-line therapy with glucocorticoids (intravenous methylprednisolone pulses followed by oral glucocorticoids) and/or plasma exchanges failed to achieve the treatment target: a reduction of ≥3 points in the global ESSDAI score. This protocol addresses that unmet need.
Evidence supports escalation to an intravenous B-cell depleting biologic therapy, administered in two infusions. The complete regimen — including timing, sequencing, and monitoring parameters — is detailed in the full protocol.
A reduction of ≥3 points in the global ESSDAI score, reflecting a meaningful and measurable decrease in systemic disease activity.
DOI: 10.1136/annrheumdis-2019-216114
Demyelinating disease with motor deficit; cerebral vasculitis presenting with focal deficit; myelitis; meningoencephalitis.
The use of rituximab may be considered in patients with severe, refractory systemic disease.
Rituximab: rituximab 1 g/15 days (x2).
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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