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Valvular injury in a patient with PR3-ANCA-associated glomerulonephritis

Nature Clinical Practice Nephrology volume 4, pages 576–582 (2008)Cite this article

Abstract

Background An 11-year-old boy who had hematuria at a routine health check-up was later diagnosed with proteinase 3 (PR3) antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. Despite treatment with corticosteroids and immunosuppressants, he went on to develop end-stage renal disease. The patient received a renal transplant at the age of 16 years, but relapse of PR3-ANCA-related nephritis to the graft occurred three times. Each relapse was successfully treated with corticosteroids and immunosuppressants. An echocardiogram at the age of 19 years revealed moderate-to-severe aortic regurgitation. The patient died of pneumonia when he was 24 years old. Autopsy revealed a perforation in the noncoronary cusp of the aortic valve and recurrence of crescentic glomerulonephritis in the transplanted kidney.

Investigations Physical examinations, urine and blood analyses, renal biopsies, echocardiograms and autopsy.

Diagnosis PR3-ANCA-associated glomerulonephritis, recurrence of crescentic glomerulonephritis to the graft, aortic regurgitation and perforation in the noncoronary cusp of the aortic valve.

Management Immunosuppressants and corticosteroids.

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Figure 1: Images of the aortic valve in the main patient presented.
Figure 2: Images of the cardiac valves in a second patient with PR3-ANCA-associated glomerulonephritis (see Box 1).

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Acknowledgements

This paper was supported in part by a Health and Labor Science Research Grant from the Japanese Ministry of Health, Labor and Welfare.

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Authors and Affiliations

  1. H Iwatani is a Nephrology Fellow at Osaka Kosei-Nenkin Hospital, Y Nagasawa is an Assistant Professor of Nephrology at Osaka University Hospital, K Oka is Director of Pathology at Osaka Kaisei Hospital, Y Isaka is Associate Professor of Advanced Technology for Transplantation and Nephrology at Osaka University Graduate School of Medicine, and E Imai is Clinical Professor of Nephrology at Osaka University Hospital, Osaka, Japan.,

    Hirotsugu Iwatani, Yasuyuki Nagasawa, Kazumasa Oka, Yoshitaka Isaka & Enyu Imai

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  1. Hirotsugu Iwatani
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Correspondence to Enyu Imai.

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Iwatani, H., Nagasawa, Y., Oka, K. et al. Valvular injury in a patient with PR3-ANCA-associated glomerulonephritis. Nat Rev Nephrol 4, 576–582 (2008). https://doi.org/10.1038/ncpneph0943

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