Document Detail

Systemic granulomatous necrotizing vasculitis in a MPO-ANCA-positive patient.
MedLine Citation:
PMID:  15260856     Owner:  NLM     Status:  MEDLINE    
We present a case of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis that demonstrated a systemic granulomatous lesion at autopsy. The patient initially showed anorexia, general malaise and anemia. Colon fiber was examined to detect the bleeding site, which revealed ischemic mucosal damage associated with venous fibrin thrombus. Because a high titer of MPO-ANCA was found, ANCA-associated vasculitis was suspected and the patient was started on steroid pulse therapy. However, anemia, renal failure and respiratory failure worsened and the patient died of sudden cardiac failure 2 days after the start of the therapy. An autopsy revealed systemic arteritis in multiple organs including the kidneys, liver, spleen, gastrointestinal system and genital organs that indicated fibrinoid necrosis accompanied by granulomatous reaction with multinucleated giant cells; the granulomatous reaction further extended along the splenic capsule. Glomerulonephritis and diffuse pulmonary damage, which are common in MPO-ANCA-associated vasculitis, were almost absent but parapleural fibrosis was present. The direct cause of death was presumed to be hemorrhagic shock due to rupture of an aneurysm in the gastric subserosa. As far as we know, this is the first case of a systemic granulomatous reaction in MPO-ANCA-positive vasculitis, although the cause of the granulomatous lesion is unknown.
Atsushi Kurata; Yu Nishimura; Tsunee Yamato; Yoshihiro Arimura; Akira Yamada; Atsuhiko Sakamoto; Yasunori Fujioka
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pathology international     Volume:  54     ISSN:  1320-5463     ISO Abbreviation:  Pathol. Int.     Publication Date:  2004 Aug 
Date Detail:
Created Date:  2004-07-20     Completed Date:  2005-01-25     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9431380     Medline TA:  Pathol Int     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  636-40     Citation Subset:  IM    
Department of Pathology, Kyorin University School of Medicine, Tokyo, Japan.
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MeSH Terms
Antibodies, Antineutrophil Cytoplasmic / immunology*
Fatal Outcome
Granuloma / complications,  immunology,  pathology*
Peroxidase / immunology*
Vasculitis / complications,  immunology,  pathology*
Reg. No./Substance:
0/Antibodies, Antineutrophil Cytoplasmic; EC

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