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. 2015 May;4(1):85-89.
doi: 10.1007/s13730-014-0145-y. Epub 2014 Sep 18.

Two brothers with collagenofibrotic glomerulopathy

Takafumi Aoki  1 , Kazuyuki Hayashi  2 , Takatoshi Morinaga  3 , Hidetaka Tomida  1 , Manabu Hishida  1 , Satoko Yamamoto  4 , Nobuyuki Kajiwara  2 , Hirofumi Tamai  1
Affiliations

Two brothers with collagenofibrotic glomerulopathy

Takafumi Aoki et al. CEN Case Rep. 2015 May.

Abstract

Collagenofibrotic glomerulopathy is a rare glomerular disease characterized by extensive accumulation of atypical type III collagen fibers within the mesangial matrix and subendothelial space. Laboratory evaluation of this disease shows a marked increase in serum procollagen III peptide (P III P) levels. Here, we report the case of two brothers with collagenofibrotic glomerulopathy confirmed by histology. Patient 1 presented with proteinuria and hypertension and patient 2 presented with nephrotic-range proteinuria. Immunohistochemistry revealed strong staining for antibodies to type III collagen in the widened subendothelial spaces in both patients. Electron microscopy revealed numerous collagenous fibers in the mesangium and subendothelial space. P III P levels were elevated in both patients. Most reported cases of collagenofibrotic glomerulopathy, including the adult-onset type, have been sporadic. Within the limits of our literature search, this is only the third report of adult siblings with collagenofibrotic glomerulopathy confirmed by histology. This report indicates that it may be beneficial to measure serum P III P levels in the siblings of patients diagnosed with adult-onset collagenofibrotic glomerulopathy.

Keywords: Collagen type III glomerulopathy; Collagenofibrotic glomerulopathy; Familial nephropathy; Procollagen III peptide; Type III collagen.

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Figures

Fig. 1
Fig. 1
Light microscopy with periodic acid–Schiff staining; ×400. The images show a glomerulus with thick capillary walls and subendothelial accumulation of pale staining homogeneous material, which is also observed throughout the mesangial regions (patient 1)
Fig. 2
Fig. 2
A double-contour appearance with periodic acid methenamine silver; ×400 (a patient 1; b patient 2)
Fig. 3
Fig. 3
Immunohistochemical staining; ×400. Positive staining in the mesangium and subendothelial space using antibodies to type III collagen (patient 1)
Fig. 4
Fig. 4
Electron microscopy using tannic acid staining; ×10000 (patient 1), marked accumulation of fibrillar substances in the mesangial area and subendothelial space. Characteristic periodicity of collagen fibers is observed
See this image and copyright information in PMC

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