利妥昔单抗治疗反应性复发缓解型 IgG4 抗接触蛋白 1 慢性炎症性脱髓鞘性多发神经根神经病合并膜性肾病：病例描述及简要复习。
Rituximab Responsive Relapsing-Remitting IgG4 Anticontactin 1 Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Membranous Nephropathy: A Case Description and Brief Review.
机构信息
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.
出版信息
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。
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