Microscopic Polyangiitis Presenting as Membranoproliferative Glomerulonephritis

Min Jeong Jeong; Tae Wook Kim; Jun Young Im; Jae Sick Jeong; Mi Ran Park; Tae Woo Kim; Choong Won Lee

doi:10.4078/jkra.2010.17.3.321

Journal List > J Korean Rheum Assoc > v.17(3) > 1003746

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Jeong, Kim, Im, Jeong, Park, Kim, and Lee: Microscopic Polyangiitis Presenting as Membranoproliferative Glomerulonephritis

Original Article

J Korean Rheum Assoc 2010;17(3):321-325.

Published online: 23 September 2010

DOI: https://doi.org/10.4078/jkra.2010.17.3.321

Microscopic Polyangiitis Presenting as Membranoproliferative Glomerulonephritis

Min Jeong Jeong, Tae Wook Kim, Jun Young Im, Jae Sick Jeong, Mi Ran Park, Tae Woo Kim, Choong Won Lee

Department of Internal Medicine, Wallace Memorial Baptist Hospital, Busan, Korea

Received 3 May 201021 June 2010 Accepted 21 June 2010

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Microscopic polyangiitis (MPA) is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. The kidney is the most often affected organ in the majority of patients with MPA, and renal manifestations are usually the first symptoms. Glomerular capillaries are affected most often, resulting in necrotizing glomerulonephritis, usually in a crescent formation, with no or few immune deposits able to be demonstrated at the sites of vasculitis and glomerulonephritis. We report a case of microscopic polyangiitis in both legs with pitting edema in a 50-year-old female. Laboratory findings showed hematuria, proteinuria, and a positive peripheral antineutrophil cytoplasmic antibody. A renal biopsy revealed pauci-immune splitting and necrotizing capillary loop walls necrotizing vasculitis and membranoproliferative glomerulonephritis (MPGN). With a diagnosis of MPA, she has been managed with high dose steroid and cyclophosphamide. To our knowledge, this is the first reported case of MPA with MPGN.

Keywords: Microscopic polyangiitis, Membranoproliferative glomerulonephritis

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Fig. 1.

Glomeruli show lobular accentuation of capillary tufts, an increase in the number of mesangial cells and matrix, and occasional splitting of capillary loop walls. Small arteries show vasculitis (light microscopy, hematoxylin and eosin, ×200).

Fig. 2.

The glomerular basement membranes are thickened with subendothelial immune complex deposits. No subepithelial immune complex deposits are seen. Immunofluorescence revealed C3 deposition (electron microscopy, immunofluorescence, ×100).

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