title: Clinical Features of Idiopathic Interstitial Pneumonia with Systemic Sclerosis-Related Autoantibody in Comparison with Interstitial Pneumonia with Systemic Sclerosis.
abstract: BACKGROUND
  Patients with idiopathic interstitial pneumonias sometimes have a few features of connective tissue disease (CTD) and yet do not fulfil the diagnostic criteria for any specific CTD.
  
  
  OBJECTIVE
  This study was conducted to elucidate the characteristics, prognosis, and disease behavior in patients with interstitial lung disease (ILD) associated with systemic sclerosis (SSc)-related autoantibodies.
  
  
  METHODS
  We retrospectively analyzed medical records of 72 ILD patients: 40 patients with SSc (SSc-ILD) and 32 patients with SSc-related autoantibody-positive ILD but not with CTD (ScAb-ILD), indicating lung-dominant CTD with SSc-related autoantibody.
  
  
  RESULTS
  Patients with SSc-ILD were predominantly females and non-smokers, and most had nonspecific interstitial pneumonia confirmed by high-resolution computed tomography (HRCT) and pathological analysis. However, about half of the patients with ScAb-ILD were male and current or ex-smokers. On HRCT analysis, honeycombing was more predominant in patients with ScAb-ILD than with SSc-ILD. Pathological analysis showed the severity of vascular intimal or medial thickening in the SSc-ILD patients to be significantly higher than that in the ScAb-ILD patients. Survival curves showed that the patients with ScAb-ILD had a significantly poorer outcome than those with SSc-ILD.
  
  
  CONCLUSIONS
  Data from this study suggest that lung-dominant CTD with SSc-related autoantibody is a different disease entity from SSc-ILD.
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.|Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan.
authors: Hideaki
authors: H
authors: Yamakawa
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors: Eri
authors: E
authors: Hagiwara
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.|Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan.
authors: Hideya
authors: H
authors: Kitamura
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.|Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan.
authors: Yumie
authors: Y
authors: Yamanaka
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors: Satoshi
authors: S
authors: Ikeda
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors: Akimasa
authors: A
authors: Sekine
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors: Tomohisa
authors: T
authors: Baba
authors: Department of Radiology, Yokohama Rousai Hospital for Labour Welfare Corporation, Yokohama, Japan.
authors: Shinichiro
authors: S
authors: Iso
authors: Department of Pathobiology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors: Koji
authors: K
authors: Okudela
authors: Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors: Tae
authors: T
authors: Iwasawa
authors: Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan.
authors: Tamiko
authors: T
authors: Takemura
authors: Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan.
authors: Kazuyoshi
authors: K
authors: Kuwano
authors: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors: Takashi
authors: T
authors: Ogura
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D005260:Female; D006801:Humans; D054988:Idiopathic Interstitial Pneumonias / Q000276:immunology* / Q000503:physiopathology*; D054990:Idiopathic Pulmonary Fibrosis / Q000276:immunology / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000276:immunology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000276:immunology / Q000503:physiopathology; D014797:Vital Capacity / Q000502:physiology
chemical_list: D001323:Autoantibodies