Fatal cardiac small-vessel involvement in ANCA-associated vasculitis: an autopsy case report.
An 80-year-old Japanese man, who had fever and generalized fatigue not improved by antibiotics, was admitted to our hospital. Laboratory data indicative of renal dysfunction and antineutrophil cytoplasmic antibody (ANCA) in the serum led to the consideration of ANCA-associated vasculitis as a differential diagnosis. However, before the diagnostic confirmation, he was found dead on the bed. Autopsy revealed necrotizing crescentic glomerulonephritis in the kidneys. In addition, necrotizing granulomatous vasculitis with infiltration of multinucleated giant cells and neutrophils but not eosinophils was present in multiple organs. The direct cause of death was presumed as cardiac arrest by lethal arrhythmia because vasculitic lesions were distributed widely in the cardiac walls, acute congestion was observed in the systemic organs, and other causes of death were ruled out. This report presents the unusual manifestation of cardiac small-vessel involvement in ANCA-associated vasculitis related to sudden death.
Department of Pathology, KKR Sapporo Medical Center, Sapporo, Japan.
Sari
S
Iwasaki
Department of Pathology, KKR Sapporo Medical Center, Sapporo, Japan.
Akira
A
Suzuki
Department of Pathology, KKR Sapporo Medical Center, Sapporo, Japan.
Takashi
T
Fujisawa
Department of Cardiology, KKR Sapporo Medical Center, Sapporo, Japan.
Taiju
T
Sato
Department of Cardiology, KKR Sapporo Medical Center, Sapporo, Japan.
Shinya
S
Shirai
Department of Cardiology, KKR Sapporo Medical Center, Sapporo, Japan.
Mitsunori
M
Kamigaki
Department of Pathology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Noriyuki
N
Otsuka
Department of Pathology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Utano
U
Tomaru
Faculty of Health Sciences, Hokkaido University, Sapporo, Japan. Electronic address: aishizu@med.hokudai.ac.jp.
Akihiro
A
Ishizu
D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000473:pathology*; D001344:Autopsy; D001706:Biopsy; D002423:Cause of Death; D003324:Coronary Artery Disease / Q000209:etiology / Q000473:pathology*; D003331:Coronary Vessels / Q000473:pathology*; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D008297:Male
ANCA-associated vasculitis; Autopsy; Cardiac small-vessel involvement; Sudden death