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A case of microscopic polyangiitis associated with aortic valve insufficiency

Title
A case of microscopic polyangiitis associated with aortic valve insufficiency
Author
김태환
Keywords
Microscopic polyangiitis; Aortic valve insufficiency; Anti-neutrophil cytoplasmic antibody; Vasculitis
Issue Date
2013-04
Publisher
Springer
Citation
Rheumatology International, 2013, 33(4), P.1055-1058
Abstract
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.
URI
https://link.springer.com/article/10.1007%2Fs00296-010-1639-3http://repository.hanyang.ac.kr/handle/20.500.11754/71083
ISSN
0172-8172
DOI
10.1007/s00296-010-1639-3
Appears in Collections:
COLLEGE OF MEDICINE[S](의과대학) > MEDICINE(의학과) > Articles
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