title: Systemic granulomatous necrotizing vasculitis in a MPO-ANCA-positive patient.
abstract: 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.
authors.affiliation: Department of Pathology, Kyorin University School of Medicine, Tokyo, Japan. akurata@kyorin-u.ac.jp
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kurata
authors.forename: Yu
authors.initials: Y
authors.lastname: Nishimura
authors.forename: Tsunee
authors.initials: T
authors.lastname: Yamato
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Arimura
authors.forename: Akira
authors.initials: A
authors.lastname: Yamada
authors.forename: Atsuhiko
authors.initials: A
authors.lastname: Sakamoto
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Fujioka
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D017809:Fatal Outcome; D005260:Female; D006099:Granuloma / Q000150:complications / Q000276:immunology / Q000473:pathology*; D006801:Humans; D009336:Necrosis; D009195:Peroxidase / Q000276:immunology*; D014657:Vasculitis / Q000150:complications / Q000276:immunology / Q000473:pathology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase