Sjögren Syndrome with Meningoencephalitis: What to Do When Glucocorticoids Are Not Enough

In Sjögren syndrome, CNS involvement — including meningoencephalitis — can occur as part of active systemic disease. When initial glucocorticoid therapy fails to achieve the required level of systemic disease control, a defined next therapeutic step exists.

Clinical Scenario

This protocol applies to patients with Sjögren syndrome presenting with meningoencephalitis in the context of active systemic disease — a recognised form of CNS involvement in primary Sjögren syndrome.

Prior Treatment — Insufficient Response

Glucocorticoids were employed as the first therapeutic line for CNS meningoencephalitis. This protocol is indicated when glucocorticoid therapy has not achieved a reduction of ≥3 points in the global ESSDAI score — the accepted threshold for therapeutic response in systemic Sjögren syndrome.

Next-Line Treatment

Escalation to a specific immunosuppressive agent is the recommended approach. Which agent and how to select among the available options are detailed in the full protocol.

Goal: ≥3-point reduction in 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 lack of head-to-head studies comparing the efficacy and safety profile of immunosuppressive agents in primary SjS-2002 (leflunomide, methotrexate, azathioprine, mycophenolate, cyclophosphamide) does not permit a recommendation on the use of one agent over another.

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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