Sjögren Syndrome with Peripheral Multineuritis and Active Systemic Disease When Glucocorticoids Have Not Achieved Treatment Targets

This protocol applies to patients with Sjögren syndrome presenting with peripheral multineuritis in the context of active systemic disease, where a prior course of glucocorticoid therapy has not produced the required degree of clinical improvement.

Clinical Scenario

Peripheral multineuritis in Sjögren syndrome — which may involve severe motor deficit, ataxia, or cryoglobulinemic-related forms — requires treatment matched to its ESSDAI-defined severity. The systemic component is active, and the prior therapeutic response was insufficient to meet the accepted threshold for disease control.

Previous Treatment — Insufficient Response

An initial regimen of glucocorticoids (oral or intravenous methylprednisolone pulses) failed to achieve a reduction of ≥3 points in the global ESSDAI score — the accepted threshold for treatment response in systemic Sjögren syndrome. This protocol describes the escalation step taken after that failure.

Next-Line Approach (Partial Overview)

The escalation step involves an intravenous biologic therapy. Further options and the complete therapeutic approach — including sequencing and all supporting measures — are detailed in the full protocol.

Treatment goal: a reduction of ≥3 points in the global ESSDAI score. The full regimen is available via the access link below.

Instant Access to Structured Evidence-Based Regimens

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.
The use of rituximab may be considered in patients with severe, refractory systemic disease, and that the best indication is probably for symptoms linked to cryoglobulinemic-associated vasculitis.
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 ↗