Ito H, Moriki T, Nishiya K, Yasuoka N, Chijiwa T, Nakamura T, Arii K, Hisakawa N, Hashimoto K, Uesugi N, Takebayashi S
Second Department of Internal Medicine, Kochi Medical School, Japan.
Clin Nephrol. 1997 Nov;48(5):321-6.
We report a patient with unusual glomerulonephritis. A 24-year-old Japanese female was hospitalized in October 1995 because of nephrotic syndrome. Lobular form glomerulonephritis with mesangial proliferation associated with massive wide-spread accumulation of slightly eosinophilic, periodic acid Schiff-positive amorphous materials in the luminal side of the capillary walls and paramesangial area was observed in the renal biopsy specimen. Immunofluorescent study revealed massive strong staining for IgM and C4 along the capillary walls and in the mesangium. Deposits of IgA, IgG, C3 and fibrinogen were also observed. Electron microscopy showed normal thickness of the capillary basement membrane and a large amount of subendothelial and paramesangial electron dense, finely granular deposits without fibrils or tubular structures. There were no clinical or laboratory findings of systemic diseases, such as systemic lupus erythematosus and cryoglobulinemia. Therefore, we believed that this case involved an unusual idiopathic glomerular disease with massive subendothelial and paramesangial immune deposits. Glomerulonephritis in this patient appeared to be resistant to treatment with corticosteroids and that this glomerulopathy may be a progressive disease as shown during the 3-year observation. Furthermore, our patient had idiopathic hyperprolactinemia and subclinical hypothyroidism. However, the relationship between glomerulonephritis and endocrinopathy in our patient is unknown.
我们报告一例患有罕见肾小球肾炎的患者。一名24岁的日本女性于1995年10月因肾病综合征住院。肾活检标本显示为小叶型肾小球肾炎伴系膜增生，毛细血管壁腔侧和系膜旁区有大量广泛堆积的轻度嗜酸性、过碘酸希夫染色阳性的无定形物质。免疫荧光研究显示沿毛细血管壁和系膜有大量IgM和C4强染色。还观察到IgA、IgG、C3和纤维蛋白原的沉积。电子显微镜显示毛细血管基底膜厚度正常，大量内皮下和系膜旁电子致密、细颗粒状沉积物，无纤维或管状结构。没有系统性疾病的临床或实验室检查结果，如系统性红斑狼疮和冷球蛋白血症。因此，我们认为该病例涉及一种罕见的特发性肾小球疾病，伴有大量内皮下和系膜旁免疫沉积物。该患者的肾小球肾炎似乎对皮质类固醇治疗耐药，并且这种肾小球病可能是一种进行性疾病，如3年观察期间所示。此外，我们的患者患有特发性高催乳素血症和亚临床甲状腺功能减退。然而，我们患者的肾小球肾炎与内分泌病之间的关系尚不清楚。
