TY - JOUR
TI - Mortality in systemic sclerosis: an international meta-analysis of
individual patient data
AU - Ioannidis, JPA
AU - Vlachoyiannopoulos, PG
AU - Haidich, AB
AU - Medsger,
AU - TA
AU - Lucas, M
AU - Michet, CJ
AU - Kuwana, M
AU - Yasuoka, H
AU - van den
AU - Hoogen, F
AU - Boome, LT
AU - van Laar, JM
AU - Verbeet, NL and
AU - Matucci-Cerinic, M
AU - Georgountzos, A
AU - Moutsopoulos, HM
JO - AMERICAN JOURNAL OF MEDICINE
PY - 2005
VL - 118
TODO - 1
SP - 2-10
PB - EXCERPTA MEDICA INC-ELSEVIER SCIENCE INC
SN - 0002-9343
TODO - 10.1016/j.amjmed.2004.04.031
TODO - systemic sclerosis; mortality; predictive model; meta-analysis; cohort
TODO - Purpose: 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.
Methods: 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. ne 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).
Results: Among 1645 incident cases, 578 deaths occurred over 11,521
person-years of follow-up. mortality ratios varied by cohort.(1.5 to
7.2). In multivariate analyses that adjusted for age and Standardized m
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. Analysis that included all cases in each center (n = 3311;
total follow-up: 19,990 person-years) yielded larsel similar results.
Conclusion: 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. 0 2005 Elsevier Inc. All rights reserved.
ER -