Clin Exp Nephrol. 2010 Jun;14(3):294-5. doi: 10.1007/s10157-009-0259-2. Epub 2010 Jan 6.
A 40-year-old male developed swallowing difficulties, loss of strength, and imbalance. On admission, the patient exhibited bifacial, extremity weakness, ataxia, impaired sensation, and areflexia. Electrophysiology and nerve biopsy suggested demyelination. Spinal fluid revealed increased protein content. Plasmapheresis showed benefit, but neuropathy relapsed. At second recurrence, urine analysis showed heavy proteinuria. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS). Methylprednisolone and oral cyclophosphamide were given. Long-term steroids and immunoglobulin showed steady benefit. Concurrence of chronic inflammatory demyelinating polyneuropathy and FSGS suggests synergistic cellular and humoral autoimmune mechanisms related to either cross-reactivity within antigenic targets or mimicry between neural and renal epitopes.
一位 40 岁男性出现吞咽困难、无力和失衡。入院时,患者表现为面部、四肢无力、共济失调、感觉减退和反射消失。电生理学和神经活检提示脱髓鞘。脑脊液显示蛋白含量增加。血浆置换显示有效,但神经病再次复发。第二次复发时,尿液分析显示大量蛋白尿。肾活检显示局灶节段性肾小球硬化症(FSGS)。给予甲基强的松龙和口服环磷酰胺。长期使用类固醇和免疫球蛋白显示出稳定的疗效。慢性炎症性脱髓鞘性多发性神经病和 FSGS 的并发提示与抗原靶点内的交叉反应或神经和肾表位之间的模拟相关的协同细胞和体液自身免疫机制。