title: Presence of Citrullinated Histone H3-Positive Neutrophils in Microscopic Polyangiitis from the Early Phase: An Autopsy Proven Case.
abstract: A 76-year-old man was admitted with general fatigue, weight loss, fever, headache, renal failure, and a high serum level of myeloperoxidase-antineutrophil cytoplasmic antibody. Biopsy revealed citrullinated histone H3 (citH3)-positive neutrophils adherent to the temporal artery endothelium. Three days after completing pulse steroid therapy, he suffered from a sudden disturbance of consciousness and died. On autopsy, the kidneys showed the most severe vasculitis with dense infiltration of citH3-positive neutrophils. The lungs showed intra-alveolar hemorrhage due to capillaritis. Severe brain hemorrhage was found in the left frontal lobe and putamen with uncal herniation. No vasculitis or thrombi was observed in the brain. The right dura mater was thickened due to fibrosis and inflammation. In conclusion, autopsy revealed systemic vasculitis with infiltration of abundant citH3-positive neutrophils, suggesting that the neutrophil extracellular trap formation and citH3 might play important roles in the early phases and development of microscopic polyangiitis.
authors.affiliation: Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Matsuda
authors.affiliation: Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Hamayasu
authors.affiliation: Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Seki
authors.affiliation: Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Nonaka
authors.affiliation: Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.|Department of Pathology, Henan University of Traditional Chinese Medicine, ZhengZhou, China.
authors.forename: Tan
authors.initials: T
authors.lastname: Wang
authors.affiliation: Department of Rheumatology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
authors.forename: Takumi
authors.initials: T
authors.lastname: Matsumoto
authors.affiliation: Department of Nephrology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
authors.forename: Yoshitomo
authors.initials: Y
authors.lastname: Hamano
authors.affiliation: Department of Neuropathology (Brain Bank for Aging Research), Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Sumikura
authors.affiliation: Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan.
authors.forename: Toshio
authors.initials: T
authors.lastname: Kumasaka
authors.affiliation: Department of Neuropathology (Brain Bank for Aging Research), Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Murayama
authors.affiliation: Faculty of Health Sciences, Hokkaido University, Sapporo, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Ishizu
authors.affiliation: Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Shimizu
authors.affiliation: Department of Rheumatology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
authors.forename: Takahiko
authors.initials: T
authors.lastname: Sugihara
authors.affiliation: Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.|Research Team for Geriatric Pathology, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan.
authors.forename: Tomio
authors.initials: T
authors.lastname: Arai
mesh_terms: D000368:Aged; D001344:Autopsy; D002956:Citrulline; D065206:Extracellular Traps; D006657:Histones / Q000378:metabolism*; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000473:pathology*; D009504:Neutrophils / Q000473:pathology*
chemical_list: D006657:Histones; D002956:Citrulline
keywords: citrullinated histone H3; elderly; microscopic polyangiitis; myeloperoxidase-antineutrophil cytoplasmic antibody; neutrophil extracellular trap