title: Lung and gastrointestinal complications are leading causes of death in SCORE, a multi-ethnic Singapore systemic sclerosis cohort.
abstract: OBJECTIVE
  To assess contemporary outcomes and predictors of mortality in the well-characterized multi-ethnic systemic sclerosis cohort Singapore (SCORE).
  
  
  METHODS
  From 2008, patients diagnosed with systemic sclerosis (SSc) fulfilling the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) or Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria were recruited from three major tertiary rheumatology centres in Singapore. Mortality was verified with the Singapore National Registry of Deaths and in-hospital cause of death was determined by two independent reviewers, up to 10 December 2013. A Cox proportional hazard (PH) regression analysis was used to examine the association between demographic and clinical indices and mortality, controlling for age and race.
  
  
  RESULTS
  Of the 349 patients (86.8% female; 77.7% Chinese), 97.4% fulfilled the ACR/EULAR 2013 criteria. The mean age at diagnosis was 46.2 years. The prevalence of limited (lcSSc), diffuse (dcSSc) cutaneous SSc, and SSc-overlap syndromes was 34.4, 37.1, and 26.8%, respectively. Thirty-five patients died after a mean follow-up of 2.1 years (743.6 person-years). Fifty-seven per cent of deaths were attributed to SSc, with pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and gastrointestinal (GI) complications as the leading causes of death. Multivariate analysis (n = 275) showed that smoking [hazard ratio (HR) 4.0, 95% confidence interval (CI) 1.5-10.6], SSc-overlap (HR 6.0, 95% CI 1.8-19.1), baseline renal involvement (HR 2.5, 95% CI 1.1-6.0), pulmonary artery systolic pressure (PASP) ≥ 40 mmHg on echocardiography (HR 5.1, 95% CI 2.2-11.7), treatment for peripheral vasculopathy (HR 2.6, 95% CI 1.1-6.5), and parenteral nutrition (HR 8.8, 95% CI 2.2-34.3) were independent predictors of mortality.
  
  
  CONCLUSIONS
  PAH, ILD, and GI complications were leading causes of death in this cohort. We identified a high-risk group of patients who would benefit from closer monitoring and early intervention.
authors.affiliation: a Division of Rheumatology , University Medicine Cluster, National University Health System , Singapore , Singapore.|b Yong Loo Lin School of Medicine , National University of Singapore , Singapore.
authors.forename: A
authors.initials: A
authors.lastname: Santosa
authors.affiliation: c Saw Swee Hock School of Public Health , National University of Singapore , Singapore.
authors.forename: C S
authors.initials: CS
authors.lastname: Tan
authors.affiliation: a Division of Rheumatology , University Medicine Cluster, National University Health System , Singapore , Singapore.|b Yong Loo Lin School of Medicine , National University of Singapore , Singapore.
authors.forename: G G
authors.initials: GG
authors.lastname: Teng
authors.affiliation: d Department of Rheumatology and Immunology , Singapore General Hospital , Singapore , Singapore.
authors.forename: W
authors.initials: W
authors.lastname: Fong
authors.affiliation: a Division of Rheumatology , University Medicine Cluster, National University Health System , Singapore , Singapore.|b Yong Loo Lin School of Medicine , National University of Singapore , Singapore.
authors.forename: A
authors.initials: A
authors.lastname: Lim
authors.affiliation: e Department of Rheumatology , Allergy and Immunology, Tan Tock Seng Hospital , Singapore , Singapore.
authors.forename: W G
authors.initials: WG
authors.lastname: Law
authors.affiliation: e Department of Rheumatology , Allergy and Immunology, Tan Tock Seng Hospital , Singapore , Singapore.
authors.forename: G
authors.initials: G
authors.lastname: Chan
authors.affiliation: d Department of Rheumatology and Immunology , Singapore General Hospital , Singapore , Singapore.|f Duke-National University of Singapore , Graduate Medical School , Singapore , Singapore.
authors.forename: S C
authors.initials: SC
authors.lastname: Ng
authors.affiliation: d Department of Rheumatology and Immunology , Singapore General Hospital , Singapore , Singapore.|f Duke-National University of Singapore , Graduate Medical School , Singapore , Singapore.
authors.forename: Ahl
authors.initials: A
authors.lastname: Low
mesh_terms: D000328:Adult; D044466:Asian People; D002423:Cause of Death; D015331:Cohort Studies; D005260:Female; D005767:Gastrointestinal Diseases / Q000209:etiology* / Q000401:mortality; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality; D017563:Lung Diseases, Interstitial / Q000209:etiology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality*; D012846:Singapore / Q000453:epidemiology