This protocol addresses patients with Sjögren syndrome who present concurrently with neuromyelitis optica spectrum disorder (NMOSD) and evidence of active systemic disease. This is a first-line treatment approach for this specific clinical combination.
Neurological manifestations in this setting may include demyelinating disease with motor deficit, myelitis, cerebral vasculitis presenting with focal deficit, and meningoencephalitis — reflecting a high-severity phenotype that requires timely, targeted management.
Management in this setting involves glucocorticoid therapy. The structured protocol specifies the sequence, route of administration, and transition strategy — including when intravenous pulse administration is indicated — at the minimum dose and duration necessary to control active disease.
The clinical goal is meaningful reduction in systemic disease activity, as defined by:
Reduction of ≥3 points in the global ESSDAI scoreDemyelinating 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.
GC (recommended dose in mg/kg/day); short-term course whenever possible; consider methylprednisolone pulses in severe cases.
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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