title: An autopsy-proven case of myeloperoxidase-antineutrophil cytoplasmic antibody-positive polyarteritis nodosa with acute renal failure and alveolar hemorrhage.
abstract: An 80-year-old woman positive for myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) was admitted with a 3-month history of fever, general malaise, and weight loss, after unsuccessful treatment with antibiotics. Upon admission, her fever persisted, and there was concomitant deterioration of renal function without active urine sediments. Furthermore, she developed hemoptysis, and chest computed tomography (CT) scan revealed bilateral diffuse alveolar hemorrhage. Although a renal biopsy was not performed because of her dementia, we initially suspected microscopic polyangiitis (MPA) on the basis of her clinical course. Because of her poor general condition, she was administered a low dose of prednisolone. Although her fever subsided, she suffered from intractable alveolar hemorrhage and eventually died from respiratory failure. During the autopsy, fibrinoid necrosis was restricted to medium-sized arteries, including the arcuate arteries of the kidneys and the bronchial arteries, without necrotizing crescentic glomerulonephritis and alveolar capillaritis. Therefore, polyarteritis nodosa (PAN) was diagnosed. It is important to distinguish between MPA and PAN because they can lead to life-threatening complications, and their treatment strategies and prognosis are different. When a patient presents with MPO-ANCA, alveolar hemorrhage, and acute renal failure with little evidence of glomerulonephritis, a differential diagnosis of PAN should be made; however, it is difficult to do so without pathological findings. Therefore, pathological examination should be carried out whenever possible.
authors.affiliation: Department of Kidney Disease and Hypertension, Osaka General Medical Center, 3-1-56 Bandaihigashi, Sumiyoshi-ku, Osaka 558-8558, Japan. yusuke7771@gmail.com
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Sakaguchi
authors.forename: Takuya
authors.initials: T
authors.lastname: Uehata
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Kawabata
authors.forename: Kakuya
authors.initials: K
authors.lastname: Niihata
authors.forename: Akihiro
authors.initials: A
authors.lastname: Shimomura
authors.forename: Akira
authors.initials: A
authors.lastname: Suzuki
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Kaneko
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Shoji
authors.forename: Kohki
authors.initials: K
authors.lastname: Shimazu
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Fushimi
authors.forename: Yoshiharu
authors.initials: Y
authors.lastname: Tsubakihara
mesh_terms: D058186:Acute Kidney Injury / Q000276:immunology / Q000473:pathology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D005260:Female; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D008171:Lung Diseases / Q000473:pathology; D055953:Microscopic Polyangiitis / Q000175:diagnosis; D009195:Peroxidase / Q000276:immunology*; D010488:Polyarteritis Nodosa / Q000175:diagnosis / Q000473:pathology*; D011650:Pulmonary Alveoli / Q000473:pathology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase