Treatment of Sjögren Syndrome with Neuromyelitis Optica Spectrum Disorder After Glucocorticoid Treatment Has Failed

This protocol applies to patients with active systemic Sjögren syndrome who have neuromyelitis optica spectrum disorder (NMOSD) as a CNS comorbidity and in whom first-line glucocorticoid therapy did not achieve the defined treatment target.

Clinical Scenario

The patient carries both Sjögren syndrome and neuromyelitis optica spectrum disorder with active systemic disease. CNS involvement in this setting may include demyelinating disease with motor deficit, myelitis, meningoencephalitis, or cerebral vasculitis presenting with focal deficit.

Previous Line Did Not Reach Target

A prior course of glucocorticoids — intravenous methylprednisolone pulses followed by oral glucocorticoids — was used for CNS involvement (NMOSD). The required treatment response was a reduction of ≥3 points in the global ESSDAI score. That target was not achieved. This protocol is the defined next step after that failure.

Next-Line Approach (Partial — Full Protocol Below)

The next-line strategy shifts to intravenous biologic therapy administered on a structured schedule. Two agent options are outlined in the protocol — agent selection, sequencing, and monitoring details are available in the complete regimen.

Treatment Target

The defined clinical goal is a reduction of ≥3 points in the global ESSDAI score.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/annrheumdis-2019-216114

Demyelinating disease with motor deficit; cerebral vasculitis presenting with focal deficit; myelitis; meningoencephalitis.

Rituximab: rituximab 1 g/15 days (x2). ABA: abatacept 0, 2, 4 weeks and every 4 weeks.

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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