A case of microscopic polyangiitis associated with aortic valve insufficiency.
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.
Division of Rheumatology, Department of Internal Medicine, Hanyang University College of Medicine, #17 Haengdang-dong, Seongdong-gu, Seoul 133-792, Korea.
Bae Keun
BK
Kim
So-Yeon
SY
Park
Chan-Bum
CB
Choi
Tae-Hwan
TH
Kim
Jae-Bum
JB
Jun
Se Min
SM
Jang
Moon Hyang
MH
Park
Wan-Sik
WS
Uhm
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001022:Aortic Valve Insufficiency / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D008875:Middle Aged
D019268:Antibodies, Antineutrophil Cytoplasmic