Early Mortality in a Multinational Systemic Sclerosis Inception Cohort Academic Article uri icon

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abstract

  • OBJECTIVE: To determine mortality and causes of death in a multinational inception cohort of subjects with systemic sclerosis (SSc). METHODS: 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. RESULTS: 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. CONCLUSION: 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

  • Hao, Yanjie
  • Hudson, Marie
  • Baron, Murray
  • Carreira, Patricia
  • Stevens, Wendy
  • Rabusa, Candice
  • Tatibouet, Solene
  • Carmona, Loreto
  • Joven, Beatriz E
  • Huq, Molla
  • Proudman, Susanna
  • Nikpour, Mandana
  • Abu‐Hakima, M
  • Baron, M
  • Cabral, AR
  • Docherty, P
  • Fortin, PR
  • Fritzler, M
  • Grodzicky, T
  • Gyger, G
  • Hudson, M
  • Jones, N
  • Kaminska, E
  • Khalidi, N
  • Larche, Margaret
  • LeClercq, S
  • Ligier, S
  • Markland, J
  • Masetto, A
  • Mathieu, J‐P
  • Pope, J
  • Robinson, D
  • Rodriguez‐Reyna, TS
  • Smith, D
  • Sutton, E
  • Thorne, C
  • Nikpour, M
  • Proudman, S
  • Roddy, J
  • Sahhar, J
  • Stevens, W
  • Walker, J
  • Youssef, P
  • Zochling, J

publication date

  • May 2017

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