Case ReportCollagenofibrotic glomerulopathy: A systemic disease
Section snippets
Case report
A 38-year-old Japanese man was admitted to a hospital because of proteinuria and ankle edema in 1976. He had diphtheria at 3 years of age. His mother had diabetes mellitus, hypertension, and ischemic heart disease. However, consanguinity was not shown. Blood pressure was 140/86 mm Hg, urinary protein was almost 5.0 g/day, and creatinine clearance was 33.0 mL/min. Renal biopsy showed unclassified glomerulopathy. Six years later (June 1982), he was admitted to our hospital to evaluate the cause
Light microscopy
Specimens from the autopsy were fixed in 10% phosphate-buffered formalin, embedded in paraffin, and sectioned at 1 to 2 μm. The sections were stained with hematoxylin and eosin, periodic acid-Shiff, Heidenhain's azocarmine anilin blue (Azan), and periodic acid silver methenamine (PASM).
Electron microscopy
Samples for electron microscopy were fixed with formalin, postfixed with 1% osmium tetraoxide, dehydrated in graded alcohols, and embedded in Epon. The sections were treated with PASM and periodic acid
Kidney
Kidney autopsy specimens showed severe cortical atrophy. Most glomeruli were globally sclerosed without patent capillary lumen. But lobulation of glomerular tufts remained and was stained strongly with PASM stain, similarly to the findings of the second specimen obtained by biopsy.18 Interstitial fibrosis and arteriolar hyalinosis of moderate degree were also found. Immunohistological examination indicated that type III collagen is present predominantly, and type I, type IV, and type VI
Discussion
The current case is completely consistent with previous reports of collagenofibrotic glomerulopathy, which is a recently defined disease entity characterized by massive accumulation of type III collagen in the glomerular mesangium and subendothelial space.17 The collagen fibrils in the glomeruli show peculiar structure, having a spiraled and frayed form. This bundled collagen is also present in nail-patella syndrome, but not in classical primary glomerulonephritis.6 There is a distinct
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2012, Laboratory InvestigationCollagen Type III Glomerulopathies
2012, Advances in Chronic Kidney DiseaseCitation Excerpt :These investigators ultimately termed the lesion collagenofibrotic glomerulopathy. It has since been documented predominantly in Japan,4-7 although at this time, reports from Europe, South America, North America, and other countries in Asia indicate that this is an uncommon disorder with worldwide presence.8-14 Although type III collagen is widely distributed as a component of the extracellular matrix, it is not found in normal glomeruli; however, it is a component of many diverse glomerular lesions as they undergo fibrosis.4
Other Glomerular Disorders and Antiphospholipid Syndrome
2010, Comprehensive Clinical Nephrology: Fourth EditionTwo Brothers in One Chinese Family With Collagen Type III Glomerulopathy
2007, American Journal of Kidney DiseasesCitation Excerpt :Collagen type III glomerulopathy is a rare renal disease with massive deposit of abnormal type III collagen peptide in glomeruli. Since 1979, there have been more than 30 cases,1-18 including 15 familial cases from 7 families reported worldwide.3,6,12,13 Of these, only 5 pairs of siblings had histological confirmation3,13 (Table 1).
Received January 21, 1998; accepted in revised form June 19, 1998.
Address reprint requests to Hirokazu Imai, MD, FJCP, Third Department of Internal Medicine Akita, University School of Medicine, 1-1-1, Hondo, 010-8543, Akita, Japan. E-mail: [email protected]