title: Early Mortality in a Multinational Systemic Sclerosis Inception Cohort.
abstract: To determine mortality and causes of death in a multinational inception cohort of subjects with systemic sclerosis (SSc).
  
  
  
  We quantified mortality as standardized mortality ratio (SMR), years of life lost, and percentage mortality in the first decade of disease. The inception cohort comprised subjects recruited within 4 years of disease onset. For comparison, we used a prevalent cohort, which included all subjects irrespective of disease duration at recruitment. We determined a single primary cause of death (SSc related or non-SSc related) using a standardized case report form, and we evaluated predictors of mortality using multivariable Cox regression.
  
  
  
  In the inception cohort of 1,070 subjects, there were 140 deaths (13%) over a median follow-up of 3.0 years (interquartile range 1.0-5.1 years), with a pooled SMR of 4.06 (95% confidence interval [95% CI] 3.39-4.85), up to 22.4 years of life lost in women and up to 26.0 years of life lost in men, and mortality in the diffuse disease subtype of 24.2% at 8 years. In the prevalent cohort of 3,218 subjects, the pooled SMR was lower at 3.39 (95% CI 3.06-3.71). In the inception cohort, 62.1% of the primary causes of death were SSc related. Malignancy, sepsis, cerebrovascular disease, and ischemic heart disease were the most common non-SSc-related causes of death. Predictors of early mortality included male sex, older age at disease onset, diffuse disease subtype, pulmonary arterial hypertension, and renal crisis.
  
  
  
  Early mortality in SSc is substantial, and prevalent cohorts underestimate mortality in SSc by failing to capture early deaths, particularly in men and those with diffuse disease.
authors.affiliation: St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia, and Peking University First Hospital, Beijing, China.
authors.forename: Yanjie
authors.initials: Y
authors.lastname: Hao
authors.affiliation: Jewish General Hospital and Lady Davis Research Institute, Montreal, Quebec, Canada.
authors.forename: Marie
authors.initials: M
authors.lastname: Hudson
authors.affiliation: Jewish General Hospital and Lady Davis Research Institute, Montreal, Quebec, Canada.
authors.forename: Murray
authors.initials: M
authors.lastname: Baron
authors.affiliation: Hospital Universitario 12 de Octubre, Madrid, Spain.
authors.forename: Patricia
authors.initials: P
authors.lastname: Carreira
authors.affiliation: St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.
authors.forename: Wendy
authors.initials: W
authors.lastname: Stevens
authors.affiliation: St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.
authors.forename: Candice
authors.initials: C
authors.lastname: Rabusa
authors.affiliation: Jewish General Hospital, Montreal, Quebec, Canada.
authors.forename: Solene
authors.initials: S
authors.lastname: Tatibouet
authors.affiliation: Instituto de Salud Musculoesqueletica, Madrid, Spain.
authors.forename: Loreto
authors.identifier: 0000-0002-4401-2551
authors.initials: L
authors.lastname: Carmona
authors.affiliation: Hospital Universitario 12 de Octubre, Madrid, Spain.
authors.forename: Beatriz E
authors.initials: BE
authors.lastname: Joven
authors.affiliation: St. Vincent's Hospital Melbourne and The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Molla
authors.initials: M
authors.lastname: Huq
authors.affiliation: Royal Adelaide Hospital and University of Adelaide, Adelaide, South Australia, Australia.
authors.forename: Susanna
authors.initials: S
authors.lastname: Proudman
authors.affiliation: St. Vincent's Hospital Melbourne and The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Mandana
authors.initials: M
authors.lastname: Nikpour
mesh_terms: D000328:Adult; D000368:Aged; D001315:Australia; D001323:Autoantibodies / Q000276:immunology; D002170:Canada; D002423:Cause of Death; D002561:Cerebrovascular Disorders / Q000401:mortality; D015331:Cohort Studies; D005260:Female; D006331:Heart Diseases / Q000209:etiology / Q000401:mortality; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality; D007410:Intestinal Diseases / Q000209:etiology / Q000401:mortality; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000209:etiology / Q000401:mortality; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009026:Mortality; D017202:Myocardial Ischemia / Q000401:mortality; D009369:Neoplasms / Q000401:mortality; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology / Q000401:mortality*; D018805:Sepsis / Q000401:mortality; D013030:Spain
chemical_list: D001323:Autoantibodies