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Presence of Citrullinated Histone H3‐Positive Neutrophils in Microscopic Polyangiitis from the Early Phase: An Autopsy Proven Case
Author(s) -
Matsuda Yoko,
Hamayasu Hideki,
Seki Atsuko,
aka Keisuke,
Wang Tan,
Matsumoto Takumi,
Hamano Yoshitomo,
Sumikura Hiroyuki,
Kumasaka Toshio,
Murayama Shigeo,
Ishizu Akihiko,
Shimizu Akira,
Sugihara Takahiko,
Arai Tomio
Publication year - 2016
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/pin.12434
Subject(s) - medicine , vasculitis , pathology , autopsy , microscopic polyangiitis , neutrophil extracellular traps , infiltration (hvac) , inflammation , physics , disease , thermodynamics
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.