Case Report
Collagenofibrotic glomerulopathy: A systemic disease

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Abstract

Collagenofibrotic glomerulopathy is a recently discovered entity that is characterized by massive accumulation of spiraled and frayed collagen fibrils in mesangial and subendothelial areas, and elevated serum levels of procollagen III peptide. We report the autopsy of a patient who received continuous ambulatory peritoneal dialysis (CAPD) therapy for 7 years. Autopsy disclosed that massive accumulation of peculiar collagen fibers was found not only in the kidney, but also in many organs including spleen, liver, myocardium, and thyroid gland. Although the possibility remains that CAPD for 7 years might change or aggravate the deposition of abnormal collagen, the current case suggests a possibility that collagenofibrotic glomerulopathy is a systemic disorder with abnormal metabolism of type III collagen.

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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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      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).

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    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]

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