Early Mortality in a Multinational Systemic Sclerosis Inception Cohort

Yanjie 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, the Australian Scleroderma Interest Group

Research output: Contribution to journalArticleResearchpeer-review

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.

Original languageEnglish
Pages (from-to)1067-1077
Number of pages11
JournalArthritis and Rheumatology
Volume69
Issue number5
DOIs
Publication statusPublished - 1 May 2017

Cite this

Hao, Y., Hudson, M., Baron, M., Carreira, P., Stevens, W., Rabusa, C., ... the Australian Scleroderma Interest Group (2017). Early Mortality in a Multinational Systemic Sclerosis Inception Cohort. Arthritis and Rheumatology, 69(5), 1067-1077. https://doi.org/10.1002/art.40027
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 ; the Canadian Scleroderma Research Group ; the Australian Scleroderma Interest Group. / Early Mortality in a Multinational Systemic Sclerosis Inception Cohort. In: Arthritis and Rheumatology. 2017 ; Vol. 69, No. 5. pp. 1067-1077.
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title = "Early Mortality in a Multinational Systemic Sclerosis Inception Cohort",
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.",
author = "Yanjie Hao and Marie Hudson and Murray Baron and Patricia Carreira and Wendy Stevens and Candice Rabusa and Solene Tatibouet and Loreto Carmona and Joven, {Beatriz E.} and Molla Huq and Susanna Proudman and Mandana Nikpour and {the Canadian Scleroderma Research Group} and {the Australian Scleroderma Interest Group} and J. Sahhar",
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Hao, Y, Hudson, M, Baron, M, Carreira, P, Stevens, W, Rabusa, C, Tatibouet, S, Carmona, L, Joven, BE, Huq, M, Proudman, S, Nikpour, M, the Canadian Scleroderma Research Group & the Australian Scleroderma Interest Group 2017, 'Early Mortality in a Multinational Systemic Sclerosis Inception Cohort' Arthritis and Rheumatology, vol. 69, no. 5, pp. 1067-1077. https://doi.org/10.1002/art.40027

Early Mortality in a Multinational Systemic Sclerosis Inception Cohort. / 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; the Canadian Scleroderma Research Group; the Australian Scleroderma Interest Group.

In: Arthritis and Rheumatology, Vol. 69, No. 5, 01.05.2017, p. 1067-1077.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Early Mortality in a Multinational Systemic Sclerosis Inception Cohort

AU - Hao, Yanjie

AU - Hudson, Marie

AU - Baron, Murray

AU - Carreira, Patricia

AU - Stevens, Wendy

AU - Rabusa, Candice

AU - Tatibouet, Solene

AU - Carmona, Loreto

AU - Joven, Beatriz E.

AU - Huq, Molla

AU - Proudman, Susanna

AU - Nikpour, Mandana

AU - the Canadian Scleroderma Research Group

AU - the Australian Scleroderma Interest Group

AU - Sahhar, J.

PY - 2017/5/1

Y1 - 2017/5/1

N2 - 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.

AB - 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.

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Hao Y, Hudson M, Baron M, Carreira P, Stevens W, Rabusa C et al. Early Mortality in a Multinational Systemic Sclerosis Inception Cohort. Arthritis and Rheumatology. 2017 May 1;69(5):1067-1077. https://doi.org/10.1002/art.40027