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• W2315097289 endingPage "e1728" @default.
• W2315097289 startingPage "e1728" @default.
• W2315097289 abstract "Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P < 0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors." @default.
• W2315097289 created "2016-06-24" @default.
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• W2315097289 date "2015-10-01" @default.
• W2315097289 modified "2023-10-17" @default.
• W2315097289 title "Registry of the Spanish Network for Systemic Sclerosis" @default.
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• W2315097289 doi "https://doi.org/10.1097/md.0000000000001728" @default.
• W2315097289 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4985378" @default.
• W2315097289 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/26512564" @default.
• W2315097289 hasPublicationYear "2015" @default.
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