原发性膜性肾病伴肌肉及周围神经损伤1例报告

Z. S. Kochoyan, O. B. Bystrova, V. Dobronravov
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引用次数: 0

摘要

原发性膜性肾病(PMN)是成人肾病综合征的典型病因。其发病机制的关键是产生针对足细胞跨膜磷脂酶A2 m型受体(anti-PLA2R)的IgG4亚类自身抗体(IgG4),然后在原位沉积上皮下免疫复合物(IC)。我们报告了一例37岁的年轻男性PMN与脱髓鞘性多神经病变和骨骼肌特发性炎性病变相关的病例,证明了igg4 -抗pla2r肾外作用的可能变异,并对诊断和分泌和细胞内磷脂酶失衡的可能机制进行了扩展分析。
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Case report of primary membranous nephropathy associated with muscle and peripheral nerve damage
   Primary membranous nephropathy (PMN) typical cause of nephrotic syndrome in adults. The key point in its pathogenesis is the production of IgG4 subclass autoantibodies (IgG4) against podocytic transmembrane phospholipase A2 M-type receptor (anti-PLA2R), followed by the deposition of subepithelial immune complexes (IC) in situ. We present a case of a 37-year-old young man with PMN associated with demyelinating polyneuropathy and idiopathic inflammatory lesions of skeletal muscles demonstrating a possible variant of extrarenal effects of IgG4-anti-PLA2R with an extended analysis of diagnostics and probable mechanisms of imbalance of secreted and intracellular phospholipases.
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