Membranous nephropathy associated with type 1 autoimmune pancreatitis and dominant glomerular IgG4 deposit.
Membranous nephropathy associated with type 1 autoimmune pancreatitis and dominant glomerular IgG4 deposit.
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DOI：
10.1007/s13730-013-0077-y
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发表时间：
2014-05
期刊：
影响因子：
1
通讯作者：
Tagawa M
中科院分区：
文献类型：
--
作者：
Sueta S;Kondo M;Matsubara T;Yasuhara Y;Akiyama S;Imai E;Amaike H;Tagawa M
我们报告一例膜性肾病合并1型自身免疫性胰腺炎。男性，58岁，表现为厌食症。检查发现胰腺头部有肿块，随后切除。病理检查显示免疫球蛋白（Ig） g4阳性浆细胞弥漫性浸润，符合1型自身免疫性胰腺炎的诊断。血清IgG4升高。手术前后他出现了肾病综合征。肾活检证实膜性肾病的诊断。免疫荧光染色显示肾小球IgG4沉积在IgG亚类中占优势。小管间质性肾炎通常是igg4相关疾病肾脏受累的主要特征，但未观察到。患者接受强的松龙和几种免疫抑制剂治疗。在治疗过程中，蛋白尿程度与血清IgG4水平相关。血清抗磷脂酶A2受体抗体阴性。这些发现和以IgG4为主的肾小球沉积提示IgG4可能在IgG4相关疾病引起的继发性膜性肾病的发病机制中发挥独特的作用。
We report a case of membranous nephropathy associated with type 1 autoimmune pancreatitis. A 58-year-old man presented with anorexia. Work-up revealed a mass in the pancreatic head, which was subsequently resected. Pathological examination showed diffuse infiltration of immunoglobulin (Ig) G4-positive plasma cells, which was compatible with the diagnosis of type 1 autoimmune pancreatitis. Serum IgG4 was elevated. He developed nephrotic syndrome around the time of the surgery. Kidney biopsy confirmed the diagnosis of membranous nephropathy. Immunofluorescent staining showed predominant glomerular IgG4 deposit among IgG subclasses. Tubulointerstitial nephritis, which is usually a dominant feature of renal involvement in IgG4-related disease, was not observed. The patient was treated with prednisolone and several immunosuppressants. During the course, the degree of proteinuria was associated with the serum IgG4 level. Serum antibody against phospholipase A2 receptor was negative. These findings together with IgG4-dominant glomerular deposit suggest that IgG4 may play a unique role in the pathogenesis of secondary membranous nephropathy caused by IgG4-related diseases.