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Two brothers with collagenofibrotic glomerulopathy

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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.

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Acknowledgments

This case report received an excellent presentation award at the 42nd Japanese Society of Nephrology Western regional meeting. We thank Norihiko Suzuki, Naoko Asano, Yuriko Sawa, Naoto Kishikawa, Masaru Kawai, Hideyo Mukai, and Yoshiko Koiso for excellent technical assistance. The authors would like to thank Enago (http://www.enago.jp) for the English language review.

Conflict of interest

All the authors have declared no competing interests to declare.

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Authors and Affiliations

  1. Department of Nephrology, Anjo Kosei Hospital, 28 Higashihirokute, Anjo-cho, Anjo City, Aichi, 446-8602, Japan

    Takafumi Aoki, Takatoshi Morinaga, Hidetaka Tomida, Manabu Hishida & Hirofumi Tamai

  2. Department of Nephrology, Ikeda City Hospital, 3-1-18 Johnan, Ikeda, Osaka, 563-8510, Japan

    Kazuyuki Hayashi & Nobuyuki Kajiwara

  3. Department of Geriatric Medicine and Nephrology, Osaka University Graduate School of Medicine, 2-2 Yamada-Oka, Suita-City, Osaka, 565-0871, Japan

    Satoko Yamamoto

Authors
  1. Takafumi Aoki
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  2. Kazuyuki Hayashi
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  3. Takatoshi Morinaga
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  4. Hidetaka Tomida
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  5. Manabu Hishida
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  6. Satoko Yamamoto
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  7. Nobuyuki Kajiwara
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  8. Hirofumi Tamai
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Correspondence to Takatoshi Morinaga.

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Aoki, T., Hayashi, K., Morinaga, T. et al. Two brothers with collagenofibrotic glomerulopathy. CEN Case Rep 4, 85–89 (2015). https://doi.org/10.1007/s13730-014-0145-y

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  • DOI: https://doi.org/10.1007/s13730-014-0145-y

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