Mortality in systemic sclerosis

An International meta-analysis of individual patient data

John P A Ioannidis, Panayiotis G. Vlachoyiannopoulos, Anna Bettina Haidich, Thomas A. Medsger, Mary Lucas, Clement J. Michet, Masataka Kuwana, Hidekata Yasuoka, Frank Van Den Hoogen, Liane Te Boome, Jacob M. Van Laar, Nicolette L. Verbeet, Marco Matucci-Cerinic, Athanasios Georgountzos, Haralampos M. Moutsopoulos

Research output: Contribution to journalArticle

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Abstract

Studies on mortality associated with systemic sclerosis have been limited by small sample sizes. We aimed to obtain large-scale evidence on survival outcomes and predictors for this disease. We performed a meta-analysis of individual patient data from cohorts recruited from seven medical centers in the United States, Europe, and Japan, using standardized definitions for disease subtype and organ system involvement. The primary outcome was all-cause mortality. Standardized mortality ratios and predictors of mortality were estimated. The main analysis was based only on patients enrolled at each center within 6 months of diagnosis (incident cases). Among 1645 incident cases, 578 deaths occurred over 11,521 person-years of follow-up. Standardized mortality ratios varied by cohort (1.5 to 7.2). In multivariate analyses that adjusted for age and sex, renal (hazard ratio [HR] = 1.9; 95% confidence interval [CI]: 1.4 to 2.5), cardiac (HR = 2.8; 95% CI: 2.1 to 3.8), and pulmonary (HR = 1.6; 95% CI: 1.3 to 2.2) involvement, and anti-topoisomerase I antibodies (HR = 1.3; 95% CI: 1.0 to 1.6), increased mortality risk. Renal, cardiac, and pulmonary involvement tended to occur together (P <0.001). For patients without adverse predictors for 3 years after enrollment, the subsequent risk of death was not significantly different from that for the general population in three cohorts, but was significantly increased in three cohorts that comprised mostly referred patients. Analyses that included all cases in each center (n = 3311; total follow-up: 19,990 person-years) yielded largely similar results. Systemic sclerosis confers a high mortality risk, but there is considerable heterogeneity across settings. Internal organ involvement and anti-topoisomerase I antibodies are important determinants of mortality.

Original languageEnglish
Pages (from-to)2-10
Number of pages9
JournalAmerican Journal of Medicine
Volume118
Issue number1
DOIs
Publication statusPublished - 2005 Jan

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Systemic Scleroderma
Meta-Analysis
Mortality
Confidence Intervals
Type I DNA Topoisomerase
Kidney
Lung
Antibodies
Sample Size
Japan
Multivariate Analysis
Survival
Population

Keywords

  • Cohort
  • Meta-analysis
  • Mortality
  • Predictive model
  • Systemic sclerosis

ASJC Scopus subject areas

  • Nursing(all)

Cite this

Ioannidis, J. P. A., Vlachoyiannopoulos, P. G., Haidich, A. B., Medsger, T. A., Lucas, M., Michet, C. J., ... Moutsopoulos, H. M. (2005). Mortality in systemic sclerosis: An International meta-analysis of individual patient data. American Journal of Medicine, 118(1), 2-10. https://doi.org/10.1016/j.amjmed.2004.04.031

Mortality in systemic sclerosis : An International meta-analysis of individual patient data. / Ioannidis, John P A; Vlachoyiannopoulos, Panayiotis G.; Haidich, Anna Bettina; Medsger, Thomas A.; Lucas, Mary; Michet, Clement J.; Kuwana, Masataka; Yasuoka, Hidekata; Van Den Hoogen, Frank; Te Boome, Liane; Van Laar, Jacob M.; Verbeet, Nicolette L.; Matucci-Cerinic, Marco; Georgountzos, Athanasios; Moutsopoulos, Haralampos M.

In: American Journal of Medicine, Vol. 118, No. 1, 01.2005, p. 2-10.

Research output: Contribution to journalArticle

Ioannidis, JPA, Vlachoyiannopoulos, PG, Haidich, AB, Medsger, TA, Lucas, M, Michet, CJ, Kuwana, M, Yasuoka, H, Van Den Hoogen, F, Te Boome, L, Van Laar, JM, Verbeet, NL, Matucci-Cerinic, M, Georgountzos, A & Moutsopoulos, HM 2005, 'Mortality in systemic sclerosis: An International meta-analysis of individual patient data', American Journal of Medicine, vol. 118, no. 1, pp. 2-10. https://doi.org/10.1016/j.amjmed.2004.04.031
Ioannidis JPA, Vlachoyiannopoulos PG, Haidich AB, Medsger TA, Lucas M, Michet CJ et al. Mortality in systemic sclerosis: An International meta-analysis of individual patient data. American Journal of Medicine. 2005 Jan;118(1):2-10. https://doi.org/10.1016/j.amjmed.2004.04.031
Ioannidis, John P A ; Vlachoyiannopoulos, Panayiotis G. ; Haidich, Anna Bettina ; Medsger, Thomas A. ; Lucas, Mary ; Michet, Clement J. ; Kuwana, Masataka ; Yasuoka, Hidekata ; Van Den Hoogen, Frank ; Te Boome, Liane ; Van Laar, Jacob M. ; Verbeet, Nicolette L. ; Matucci-Cerinic, Marco ; Georgountzos, Athanasios ; Moutsopoulos, Haralampos M. / Mortality in systemic sclerosis : An International meta-analysis of individual patient data. In: American Journal of Medicine. 2005 ; Vol. 118, No. 1. pp. 2-10.
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