Treatment of Sjögren Syndrome with Ganglionopathy or Chronic Inflammatory Demyelinating Polyradiculopathy in Active Systemic Disease

This protocol applies to patients with Sjögren syndrome who have developed ganglionopathy or chronic inflammatory demyelinating polyradiculopathy (CIDP) alongside active systemic disease. Neuropathy in this setting — including ganglionopathy and polyradiculopathies — may present with severe motor deficit or ataxia, representing a high-burden systemic manifestation that requires specific management.

The management strategy for this scenario involves an intravenous immunoglobulin-based therapy. The complete regimen, administration details, and full clinical algorithm are available in the structured protocol below.

The primary therapeutic target is a clinically meaningful reduction in systemic disease activity, assessed using the ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index).