Rituximab Responsive Relapsing–Remitting IgG4 Anticontactin 1 Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Membranous Nephropathy: A Case Description and Brief Review

Q3 Medicine Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI:10.1097/CND.0000000000000395

G. Remiche, Marta Lamartine S Monteiro, C. Catalano, J. Hougardy, E. Delmont, J. Boucraut, N. Mavroudakis

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引用次数: 1

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/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.

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慢性炎症性脱髓鞘性多根神经病变伴膜性肾病:一例描述和简要回顾

结/副结igg4相关的慢性炎症性脱髓鞘性多根神经病变(CIDP)很少涉及抗接触素(CNTN1)亚型，罕见并发肾病综合征。65岁男性出现虚弱、面瘫和平衡障碍;自愈后1个月严重复发。神经肌电图证实为CIDP。蛋白含量(462 mg/dL;N < 45)，蛋白尿(3.5 g/g肌酸)，活检证实的膜性肾病。静脉注射免疫球蛋白、皮质类固醇和血浆均不能恢复。抗cntn1免疫球蛋白G4 (IgG4)检测阳性。利妥昔单抗(375 mg/m2/周，4周)改善明显。复发缓解型抗cntn1 - cidp与肾病综合征共同发生是罕见的，它的识别是必不可少的，因为这种严重的疾病有有效的治疗方法，如利妥昔单抗。

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来源期刊

Journal of Clinical Neuromuscular Disease Medicine-Medicine (all)

CiteScore

1.60

自引率

0.00%

发文量

期刊介绍： Journal of Clinical Neuromuscular Disease provides original articles of interest to physicians who treat patients with neuromuscular diseases, including disorders of the motor neuron, peripheral nerves, neuromuscular junction, muscle, and autonomic nervous system. Each issue highlights the most advanced and successful approaches to diagnosis, functional assessment, surgical intervention, pharmacologic treatment, rehabilitation, and more.