Morita Hiroyuki, Hasegawa Takeshi, Minamoto Toshinari, Oda Yoshio, Inui Kiyoko, Tayama Hironori, Nakao Naoyuki, Nakamoto Yasushi, Ideura Terukuni, Yoshimura Ashio
Division of Nephrology, Department of Medicine, Showa University Fujigaoka Hospital, 1-30 Fujigaoka, Aoba-ku, Yokohama, Japan.
Virchows Arch. 2003 Feb;442(2):163-8. doi: 10.1007/s00428-002-0738-2. Epub 2002 Dec 13.
Collagenofibrotic glomerulopathy is considered as a form of glomerulopathy in which organized collagen type III progressively deposits. We report a case of this disease with widespread expression of collagen type V. A 65-year-old woman was admitted to our hospital for further evaluation of nephrotic-range proteinuria. The patient had had anemia and hypertension for 9 years, and proteinuria for 3 years. A renal biopsy specimen showed a remarkable mesangial expansion with Congo red-negative and periodic acid-Schiff-positive deposits. At the ultrastructural level, two forms of bundling fibers were found in the mesangium and subendothelial side of the glomerular basement membranes (GBM). The GBM itself appeared normal. Immunohistochemical investigation showed that the glomerular lesions were strongly reactive with both anti-collagen type-III and -V antibodies. Immunoelectron microscopy demonstrated collagen type V in both forms of bundling fibers. Despite therapy, her renal function declined. The clinical course and renal pathology of this case were in accordance with collagenofibrotic glomerulopathy except for the widespread expression of collagen type V. Collagen type V is a fibrillar collagen capable of forming banding fibrils. This report poses the question whether collagen type V accumulates only in this particular case or whether it is a normal component in collagenofibrotic glomerulopathy.
胶原纤维性肾小球病被认为是一种肾小球病，其中有组织的III型胶原逐渐沉积。我们报告一例该疾病伴有V型胶原广泛表达的病例。一名65岁女性因肾病范围蛋白尿进一步评估入院。患者有9年贫血和高血压病史，蛋白尿3年。肾活检标本显示系膜显著增宽，刚果红阴性、过碘酸-希夫染色阳性沉积物。在超微结构水平，在系膜和肾小球基底膜（GBM）内皮下侧发现两种形式的束状纤维。GBM本身看起来正常。免疫组织化学研究显示，肾小球病变对抗III型和V型胶原抗体均有强烈反应。免疫电镜在两种形式的束状纤维中均显示有V型胶原。尽管进行了治疗，她的肾功能仍下降。该病例的临床病程和肾脏病理符合胶原纤维性肾小球病，但V型胶原广泛表达除外。V型胶原是一种能够形成带状纤维的纤维状胶原。本报告提出了一个问题，即V型胶原是仅在这个特殊病例中积累，还是胶原纤维性肾小球病的正常成分。
