Early mortality in a multinational systemic sclerosis inception cohort

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dc.contributor.authorHao, Y.
dc.contributor.authorHudson, M.
dc.contributor.authorBaron, M.
dc.contributor.authorCarreira, P.
dc.contributor.authorStevens, W.
dc.contributor.authorRabusa, C.
dc.contributor.authorTatibouet, S.
dc.contributor.authorCarmona, L.
dc.contributor.authorJoven, B.
dc.contributor.authorHuq, M.
dc.contributor.authorProudman, S.
dc.contributor.authorNikpour, M.
dc.date.issued2017
dc.description.abstractObjective. 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 usingmultivariable 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, andmortality 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 includedmale 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.
dc.description.statementofresponsibilityYanjie Hao, Marie Hudson, Murray Baron, Patricia Carreira, Wendy Stevens, Candice Rabusa, Solene Tatibouet, Loreto Carmona, Beatriz E. Joven, Molla Huq, Susanna Proudman, Mandana Nikpour, the Canadian Scleroderma Research Group, and the Australian Scleroderma Interest Group
dc.identifier.citationArthritis & Rheumatology, 2017; 69(5):1067-1077
dc.identifier.doi10.1002/art.40027
dc.identifier.issn2326-5191
dc.identifier.issn2326-5205
dc.identifier.orcidProudman, S. [0000-0002-3046-9884]
dc.identifier.urihttp://hdl.handle.net/2440/123299
dc.language.isoen
dc.publisherWiley
dc.relation.granthttp://purl.org/au-research/grants/nhmrc/1071735
dc.rights© 2016, American College of Rheumatology
dc.source.urihttps://doi.org/10.1002/art.40027
dc.subjectCanadian Scleroderma Research Group
dc.subjectAustralian Scleroderma Interest Group
dc.titleEarly mortality in a multinational systemic sclerosis inception cohort
dc.typeJournal article
pubs.publication-statusPublished

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