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Semin Arthritis Rheum. 2014 Oct;44(2):208-19. doi: 10.1016/j.semarthrit.2014.05.010. Epub 2014 May 14.

Mortality and survival in systemic sclerosis: systematic review and meta-analysis.

Author information

1
Autoimmune Diseases Unit, Bellvitge University Hospital, Barcelona, Spain. Electronic address: mrubio@bellvitgehospital.cat.
2
Autoimmune Diseases Unit, Bellvitge University Hospital, Barcelona, Spain.
3
Vall d'Hebron University Hospital, Barcelona, Spain.

Abstract

OBJECTIVE:

To determine the mortality, survival, and causes of death in patients with systemic sclerosis (SSc) through a meta-analysis of the observational studies published up to 2013.

METHODS:

We performed a systematic review and meta-analysis of the observational studies in patients with SSc and mortality data from entire cohorts published in MEDLINE and SCOPUS up to July 2013.

RESULTS:

A total of 17 studies were included in the mortality meta-analysis from 1964 to 2005 (mid-cohort years), with data from 9239 patients. The overall SMR was 2.72 (95% CI: 1.93-3.83). A total of 43 studies have been included in the survival meta-analysis, reporting data from 13,529 patients. Cumulative survival from onset (first Raynaud's symptom) has been estimated at 87.6% at 5 years and 74.2% at 10 years, from onset (non-Raynaud's first symptom) 84.1% at 5 years and 75.5% at 10 years, and from diagnosis 74.9% at 5 years and 62.5% at 10 years. Pulmonary involvement represented the main cause of death.

CONCLUSIONS:

SSc presents a larger mortality than general population (SMR = 2.72). Cumulative survival from diagnosis has been estimated at 74.9% at 5 years and 62.5% at 10 years. Pulmonary involvement represented the main cause of death.

KEYWORDS:

Mortality; Systemic sclerosis

[Indexed for MEDLINE]

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