Sjögren Syndrome with Meningoencephalitis and Active Systemic Disease After Immunosuppressive Agents Have Not Controlled Disease

This protocol addresses patients with Sjögren syndrome who have developed meningoencephalitis as a manifestation of active systemic disease. CNS involvement at this severity — including meningoencephalitis — represents a serious, organ-threatening presentation requiring structured escalation when initial therapy has not achieved the defined treatment target.

Prior Treatment — Insufficient Response

An initial course of immunosuppressive agents (leflunomide, or methotrexate, or azathioprine, or mycophenolate, or cyclophosphamide) was the first-line approach for this level of CNS involvement. When that course does not achieve the required reduction in systemic disease activity — as measured by the global ESSDAI score — escalation to the next structured protocol is indicated.

Next-Step Approach

For patients who have not responded adequately to immunosuppressive therapy in the setting of meningoencephalitis, the structured evidence-based approach involves rituximab, a targeted biologic agent. The complete regimen — including sequencing, administration details, and monitoring guidance — is available in the full protocol.

Treatment target: 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.

The use of rituximab may be considered in patients with severe, refractory 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.

View source ↗