title: Clinical features of usual interstitial pneumonia with anti-neutrophil cytoplasmic antibody in comparison with idiopathic pulmonary fibrosis.
abstract: Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) is occasionally positive in patients with usual interstitial pneumonia (UIP). However, the differences from idiopathic pulmonary fibrosis (IPF/UIP) have not been well documented. We aimed to clarify the clinical, radiological and pathological features of UIP associated with MPO-ANCA (ANCA/UIP).
  
  
  
  We retrospectively reviewed the medical records of 12 consecutive ANCA/UIP patients not manifesting microscopic polyangiitis and 108 IPF/UIP patients with no autoantibodies, both diagnosed by surgical lung biopsy.
  
  
  
  There was no significant difference in clinical background, laboratory results and pulmonary function tests between ANCA/UIP patients and IPF/UIP patients except for the percentage of bronchoalveolar lavage neutrophils. HRCT showed subpleural reticulation in both groups. Increased attenuation around honeycombing and cysts was significantly observed in ANCA/UIP. Pathologically, ANCA/UIP had more prominent inflammatory cell infiltration, lymphoid follicles with germinal centres and cellular bronchiolitis. During the disease course, three of 12 patients (25%) developed microscopic polyangiitis. Immunosuppressive treatment tended to be more effective in ANCA/UIP patients, and the survival time in ANCA/UIP patients tended to be longer than those with IPF/UIP.
  
  
  
  ANCA/UIP may be distinguishable from IPF/UIP with a combination of HRCT findings of increased attenuation around honeycombing and cysts and some of the characteristic pathological findings. In contrast to IPF/UIP, immunosuppressive treatment could be a therapeutic option for ANCA/UIP.
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Chiaki
authors.initials: C
authors.lastname: Hosoda
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Baba
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Eri
authors.initials: E
authors.lastname: Hagiwara
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ito
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Norikazu
authors.initials: N
authors.lastname: Matsuo
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Hideya
authors.initials: H
authors.lastname: Kitamura
authors.affiliation: Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Tae
authors.initials: T
authors.lastname: Iwasawa
authors.affiliation: Department of Pathology, Graduate School of Medicine, Yokohama City University, Yokohama, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Okudela
authors.affiliation: Department of Pathology, Japan Red Cross Medical Center, Tokyo, Japan.
authors.forename: Tamiko
authors.initials: T
authors.lastname: Takemura
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ogura
mesh_terms: D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D001706:Biopsy / Q000379:methods; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007564:Japan / Q000453:epidemiology; D008168:Lung / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009195:Peroxidase / Q000032:analysis; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D012129:Respiratory Function Tests / Q000379:methods; D012189:Retrospective Studies; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D009195:Peroxidase
keywords: idiopathic pulmonary fibrosis; immunosuppressive treatment; myeloperoxidase anti-neutrophil cytoplasmic antibody; usual interstitial pneumonia