Department of Neurology, Centre de Référence Neuromusculaire, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium.
Department of Nephrology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium.
J Clin Neuromuscul Dis. 2022 Jun 1;23(4):219-226. doi: 10.1097/CND.0000000000000395.
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/m2/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.
节段性/旁正中 IgG4 相关慢性炎性脱髓鞘性多发神经病（CIDP）很少涉及抗接触蛋白（CNTN1）亚型，且异常并发肾病综合征。一位 65 岁男性出现无力、面瘫和平衡障碍；在自发恢复后，1 个月后严重复发。神经电图检查证实为 CIDP。发现蛋白血症（462mg/dL；N＜45）、蛋白尿（3.5g/g 肌酐）和活检证实的膜性肾病。静脉注射免疫球蛋白、皮质类固醇和血浆置换均未恢复。抗-CNTN1 IgG4 检测呈阳性。利妥昔单抗（375mg/m2/周，4 周）显著改善。复发性抗-CNTN1-CIDP 合并肾病综合征非常罕见，其鉴定至关重要，因为这种严重情况可采用有效的治疗方法，如利妥昔单抗。
