This protocol is for patients with Sjögren syndrome who present with axonal peripheral neuropathy alongside ongoing active systemic disease, and in whom a prior course of immunosuppressive therapy did not produce an adequate systemic response.

Clinical scenario

Axonal peripheral neuropathy in Sjögren syndrome may present with severe motor deficit or ataxia, and systemic activity is measured using ESSDAI domain scores. Management of systemic involvement is tailored to organ-specific severity as defined by ESSDAI criteria.

Prior therapy — insufficient response

Immunosuppressive agents — which may include leflunomide, methotrexate, azathioprine, mycophenolate, or cyclophosphamide — were used as the initial line. The response goal was a reduction of ≥3 points in the global ESSDAI score. When that threshold is not reached, escalation to the next protocol step is indicated.

Next-step approach (partial overview)

After failure of immunosuppressive therapy to meet the ESSDAI target, the structured protocol calls for an intravenous immunoglobulin-based regimen. The full regimen details — including administration parameters — are in the complete protocol below.

Goal: ESSDAI reduction ≥3 points

References

DOI: 10.1136/annrheumdis-2019-216114

Neuropathy (including ganglionopathy and polyradiculopathies) with severe motor deficit/ataxia; cryoglobulinemic-related multineuritis.

Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.

IVIg: intravenous immunoglobulins 0.4-2 g/kg 5 days.

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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Sjögren Syndrome with Axonal Peripheral Neuropathy When Immunosuppressive Therapy Has Not Achieved Disease Control