Rituximab Responsive Relapsing–Remitting IgG4 Anticontactin 1 Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Membranous Nephropathy: A Case Description and Brief Review. Issue 4 (June 2022)

• Record Type: Journal Article
• Authors: Remiche, Gauthier; Monteiro, Marta Lamartine S.; Catalano, Concetta; Hougardy, Jean-Michel; Delmont, Emilien; Boucraut, José; Mavroudakis, Nicolas
• Is Part Of: Journal of clinical neuromuscular disease. Volume 23:Issue 4(2022)
• Page Start: 219
• Page End: 226
• Publication Date: 2022-06
• Subjects: anticontactin -- IgG4 -- chronic inflammatory demyelinating polyradiculoneuropathy and nephrotic syndrome; Neuromuscular diseases -- Periodicals; 616.744005
• DOI: 10.1097/CND.0000000000000395
• Languages: English
• ISSNs: 1522-0443

Abstract

Abstract: Nodal/paranodal IgG4-related chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) rarely involves anticontactin (CNTN1) subtype and exceptionally complicates with nephrotic syndrome. A 65-year-old man developed weakness, facial palsy, and balance impairment; after spontaneous recovery, he severely relapsed 1 month later. Electroneuromyography confirmed CIDP. Proteinorachy (462 mg/dL; N < 45), proteinuria (3.5 g/g creatine), and biopsy-proven membranous nephropathy were identified. Intravenous immunoglobulins, corticosteroids, and plasmaphereses did not allow recovery. Anti-CNTN1 immunoglobulin G4 (IgG4) assay was positive. Rituximab (375 mg/m 2 /week, 4 weeks) provided obvious improvement. Relapsing–remitting anti–CNTN1-CIDP co-occurring with nephrotic syndrome is exceptional, and its identification is essential because efficient therapies such as rituximab are available for this severe condition.