Sjögren Syndrome with Ganglionopathy or CIDP: What to Do When Intravenous Immunoglobulins Have Not Achieved the Treatment Target
Clinical Scenario
This protocol covers patients with Sjögren syndrome who have ganglionopathy or chronic inflammatory demyelinating polyradiculopathy (CIDP) with active systemic disease, and who have not achieved the defined response goal after a course of intravenous immunoglobulins.
Neurological Involvement
Neuropathy — including ganglionopathy and polyradiculopathies — is a recognised manifestation of Sjögren syndrome in patients with active systemic disease. When present, this comorbidity shapes the treatment pathway and escalation criteria.
Prior Therapy — Insufficient Response
The preceding treatment line employed intravenous immunoglobulins. The required treatment target — a reduction of at least 3 points in the global ESSDAI score — was not reached. This insufficient response is the trigger for escalation to the current protocol.
Next-Line Approach (Partial)
For patients with severe, refractory systemic Sjögren syndrome where the initial treatment line has failed, rituximab given intravenously may be considered. The complete infusion schedule, course details, and monitoring guidance are available in the full structured regimen.
Treatment target: reduction of ≥3 points in the global ESSDAI score.
References
DOI: 10.1136/annrheumdis-2019-216114
- Neuropathy (including ganglionopathy and polyradiculopathies) with severe motor deficit/ataxia; cryoglobulinemic-related multineuritis.
- The use of rituximab may be considered in patients with severe, refractory systemic disease.
- Rituximab: rituximab 1 g/15 days (x2).
- 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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