Document Detail

A case of microscopic polyangiitis associated with aortic valve insufficiency.
MedLine Citation:
PMID:  21120499     Owner:  NLM     Status:  In-Data-Review    
Microscopic polyangiitis (MPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by inflammation of small-sized vessels. Although there have been some reports of ANCA-associated vasculitis presenting as aortitis syndrome, MPA rarely involves large-sized vessels such as the aorta. We report an unusual case of MPA combined with severe acute aortic valve insufficiency in a 56-year-old man. He initially presented with prolonged fever, skin rash, and rapidly progressive glomerulonephritis. P-ANCA and anti-myeloperoxidase (MPO) antibodies were positive, but the c-ANCA and anti-proteinase-3 antibodies were negative. Skin biopsy of the lower leg showed necrotizing arteritis. Kidney biopsy was also performed, which revealed diffuse necrotizing and crescentic glomerulonephritis (GN) consistent with pauci-immune ANCA-associated GN. Serial echocardiographic evaluations revealed aortic valve changes and worsening acute aortic valve insufficiency over a two-month period. Despite intensive treatment, our patient developed sudden cardiac arrest and died. Our patient demonstrated typical clinical features and histopathologic findings for systemic vasculitis and had a positive anti-MPO antibody, all of which were consistent with the diagnosis of MPA. Thus, MPA may have been the cause of acute aortic valve insufficiency in this case.
Bae Keun Kim; So-Yeon Park; Chan-Bum Choi; Tae-Hwan Kim; Jae-Bum Jun; Se Min Jang; Moon Hyang Park; Wan-Sik Uhm
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Publication Detail:
Type:  Journal Article     Date:  2010-12-01
Journal Detail:
Title:  Rheumatology international     Volume:  33     ISSN:  1437-160X     ISO Abbreviation:  Rheumatol. Int.     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-04-02     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8206885     Medline TA:  Rheumatol Int     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1055-8     Citation Subset:  IM    
Division of Rheumatology, Department of Internal Medicine, Hanyang University College of Medicine, #17 Haengdang-dong, Seongdong-gu, Seoul, 133-792, Korea.
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