Treatment of Sjögren Syndrome with Central Nervous System Vasculitis and Active Systemic Disease
When Sjögren syndrome presents alongside central nervous system vasculitis and evidence of active systemic disease, a specific, protocol-driven treatment approach is indicated. This scenario requires early and structured intervention aimed at measurable disease control.
Clinical scenario
This protocol addresses Sjögren syndrome complicated by CNS vasculitis with active systemic involvement. Relevant presentations in this setting include demyelinating disease with motor deficit, cerebral vasculitis with focal deficit, myelitis, and meningoencephalitis.
Treatment approach (partial overview)
The regimen for this scenario involves glucocorticoids used in combination with an immunosuppressant agent. The complete sequencing, monitoring parameters, and decision criteria are detailed in the full protocol.
Therapeutic goal
Treatment response is assessed using the global ESSDAI score, with a clinically meaningful reduction serving as the defined target for success.
References
DOI: 10.1136/annrheumdis-2019-216114
- Demyelinating disease with motor deficit; cerebral vasculitis presenting with focal deficit; myelitis; meningoencephalitis.
- GCs should be used at the minimum dose and length of time necessary to control active systemic disease.
- 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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