Treatment of Sjögren Syndrome with Myelitis in Active Systemic Disease

This protocol addresses management of Sjögren syndrome when myelitis is present as part of active systemic disease — a serious neurological manifestation requiring structured, timely clinical intervention.

Clinical Scenario

Myelitis is a recognised severe CNS manifestation in Sjögren syndrome. Its presence alongside active systemic disease signals significant disease burden and the need for targeted therapy aimed at reducing overall systemic activity.

Treatment Approach

Current evidence supports glucocorticoid-based therapy as the cornerstone of management in this setting; the full regimen, sequencing, and any additional interventions are detailed in the complete protocol.

Treatment Goal

The primary benchmark for therapeutic response is a reduction of ≥3 points in the global ESSDAI score — a validated measure of systemic disease activity in Sjögren syndrome.

ESSDAI reduction ≥ 3 points

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.
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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