title: An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis.
abstract: OBJECTIVE
  To compare survival of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) versus idiopathic pulmonary fibrosis (IPF) and patients with systemic sclerosis-associated ILD (SSc-ILD) versus other CTD-ILD followed at our center.
  
  
  METHODS
  We used the Stanford ILD database, which contains prospectively collected information on patients with ILD evaluated at our tertiary care center from 2002 to 2009. Survival at last followup from time of ILD diagnosis was calculated using the Kaplan-Meier estimator. Prognostic factors for survival in the overall cohort (IPF and CTD-ILD) and in the CTD-ILD group were identified with univariate and multivariate Cox regression models.
  
  
  RESULTS
  Of 427 patients with ILD, 148 (35%) had IPF and 76 (18%) had CTD-ILD at the baseline visit. The cumulative incidence of CTD was 4%. After a median followup of 4 years, 67 patients (36.4%) had died and 4 (2.2%) were lost to followup. Patients with IPF (n = 122) and CTD-ILD (n = 62) experienced similar survival rates (5-year survival about 50%). Patients with SSc-ILD (n = 24) experienced better survival than those with other CTD-ILD (n = 38), with 1-year, 3-year, and 5-year survival rates of 100%, 90%, and 77%, respectively, versus 78%, 42%, and 38% (p = 0.01). The presence of SSc in patients with CTD-ILD decreased the risk of death by > 80% even after correcting for age at ILD diagnosis, sex, and ethnicity (HR = 0.17, 95% CI 0.04-0.83).
  
  
  CONCLUSIONS
  Survival in patients with SSc-ILD was better than in patients with other CTD-ILD, potentially related to routine screening for and early detection of ILD in patients with SSc at our center.
authors.affiliation: Department of Medicine, Division of Immunology and Rheumatology, Division of Pulmonary and Critical Care, Stanford University School of Medicine, Palo Alto, CA, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Su
authors.forename: Mihoko
authors.initials: M
authors.lastname: Bennett
authors.forename: Susan
authors.initials: S
authors.lastname: Jacobs
authors.forename: Tessa
authors.initials: T
authors.lastname: Hunter
authors.forename: Carissa
authors.initials: C
authors.lastname: Bailey
authors.forename: Eswar
authors.initials: E
authors.lastname: Krishnan
authors.forename: Glenn
authors.initials: G
authors.lastname: Rosen
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
mesh_terms: D000328:Adult; D000368:Aged; D003240:Connective Tissue Diseases / Q000150:complications* / Q000188:drug therapy; D016208:Databases, Factual; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy / Q000401:mortality*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality*; D008875:Middle Aged; D010044:Outpatient Clinics, Hospital; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy; D015996:Survival Rate; D014481:United States
chemical_list: D007166:Immunosuppressive Agents