Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune neuropathy characterised by insidious onset, progressive course, proximal and distal symmetrical weakness, and sensory impairment. It may affect patients of any age with varying degrees of clinical involvement and response rates to existing treatments. Sjögren syndrome (SS) is a systemic autoimmune disorder that primarily affects the exocrine glands causing a sicca syndrome. It may affect the peripheral nervous system, usually causing painful small fibre or pure sensory axonal neuropathy, ganglioneuronopathy or a predominantly sensory CIDP. We report the case of a 71-year-old man diagnosed with a debilitating and difficult-to-treat CIDP who, 5 years later, developed SS with pulmonary involvement. Due to lack of response to treatments other than periodic intravenous immunoglobulin (IVIg) every 12 days, we started adjuvant treatment with rituximab which increased the time interval between IVIg therapies by 50%, providing better quality of life for the patient.
- peripheral nerve disease
- sjogren's syndrome
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Contributors The four authors had substantial contributions to the conception and design of the work. Initial drafting of the case made by RR and JM. Follow-up part drafting made by AS and FC. Important messages made by RR and JM. Revising it critically for important intellectual content made by the four authors, RR, JM, AS and FC. All four authors gave final approval of the version to be published and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy and integrity of any part of the work are appropriately investigated and resolved.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.