title: A retrospective cohort study of outcome in systemic sclerosis-associated interstitial lung disease.
abstract: BACKGROUND
  The relationship between the histological pattern and survival in systemic sclerosis-associated interstitial lung disease (SSc-ILD) is unclear. In patients with SSc-ILD, we investigated whether the clinical data obtained by non-invasive examinations could be used for prognostic evaluation, and attempted to clarify whether complicating acute exacerbation (AE) and the selection of pharmacological therapy were associated with survival.
  
  
  METHODS
  Thirty-five patients with SSc-ILD, who had not been diagnosed by surgical lung biopsy were analyzed, retrospectively. The HRCT findings were evaluated by 2 radiologists and classified into "CT-UIP" or "CT-inconsistent with UIP" patterns based on whole lung interpretations. HRCT scores were calculated based on the extent of abnormality evidenced by HRCT. The log-rank test was used to determine variables, including clinical parameters and histories.
  
  
  RESULTS
  Twelve (34%) of the 35 patients died during a median follow-up period of approximately 7.9 years. The log-rank test showed that a higher mortality was associated with higher age, a CT-UIP pattern, a higher score for ground-glass attenuation with traction bronchiectasis on HRCT, and complicating AE, whereas a lower mortality was significantly associated with the use of immunosuppressants. A CT-UIP pattern was significantly associated with a higher incidence of later AE.
  
  
  CONCLUSIONS
  Treatment with immunosuppressants was associated with a longer survival, and complicating AE is a predictor of shortened survival in SSc-ILD patients. Among the clinical parameters determined by non-invasive examinations, a CT-UIP pattern and the extent of fibrotic lesions on HRCT, but not a histological pattern of UIP, may be predictors of shortened survival.
authors.affiliation: Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: okamoto_masaki@med.kurume-u.ac.jp.
authors.forename: Masaki
authors.initials: M
authors.lastname: Okamoto
authors.affiliation: Department of Radiology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan; Center for Diagnostic Imaging, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kimichan@med.kurume-u.ac.jp.
authors.forename: Kiminori
authors.initials: K
authors.lastname: Fujimoto
authors.affiliation: Department of Radiology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan; Center for Diagnostic Imaging, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: sadojun@gmail.com.
authors.forename: Junko
authors.initials: J
authors.lastname: Sadohara
authors.affiliation: Department of Radiology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: furuya@kyumed.jp.
authors.forename: Kiyomi
authors.initials: K
authors.lastname: Furuya
authors.affiliation: Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kaieda@med.kurume-u.ac.jp.
authors.forename: Shinjiro
authors.initials: S
authors.lastname: Kaieda
authors.affiliation: Department of Internal Medicine and Rheumatology, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan. Electronic address: miyamura@kyumed.jp.
authors.forename: Tomoya
authors.initials: T
authors.lastname: Miyamura
authors.affiliation: Department of Respirology, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan. Electronic address: suematsu-e@kyumed.jp.
authors.forename: Eiichi
authors.initials: E
authors.lastname: Suematsu
authors.affiliation: Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kita-sir1028@jcom.home.ne.jp.
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Kitasato
authors.affiliation: Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kawayama_tomotaka@med.kurume-u.ac.jp.
authors.forename: Tomotaka
authors.initials: T
authors.lastname: Kawayama
authors.affiliation: Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: ida@med.kurume-u.ac.jp.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Ida
authors.affiliation: Department of Respirology, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan; Department of Clinical Research Institute, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan. Electronic address: ichiki@kyumed.jp.
authors.forename: Masao
authors.initials: M
authors.lastname: Ichiki
authors.affiliation: Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: hoshino@med.kurume-u.ac.jp.
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Hoshino
mesh_terms: D000368:Aged; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications*; D014057:Tomography, X-Ray Computed
chemical_list: D007166:Immunosuppressive Agents
keywords: Acute exacerbation; Computed tomography; Immunosuppressant; Interstitial lung disease; Systemic sclerosis