1. Registry of the Spanish Network for Systemic Sclerosis
- Author
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Simeón-Aznar, C.P., Fonollosa-Plá, V., Tolosa-Vilella, Carles, Espinosa-Garriga, G., Campillo-Grau, M., Ramos-Casals, M., García-Hernández, F.J., Castillo-Palma, M.J., Sánchez-Román, J., Callejas-Rubio, J.L., Ortego-Centeno, N., Egurbide-Arberas, M.V., Trapiellla-Martínez, L., Caminal-Montero, L., Sáez-Comet, L., Velilla-Marco, J., Camps-García, M.T., de Ramón-Garrido, E., Esteban-Marcos, E.M., Pallarés-Ferreres, L., Navarrete-Navarrete, N., Vargas-Hitos, J.A., de la Torre, R. Gómez, Salvador-Cervello, G., Rios-Blanco, J.J., and Vilardell-Tarrés, M.
- Subjects
Adult ,Male ,Scleroderma, Systemic ,Observational Study ,Middle Aged ,Risk Factors ,Spain ,Cause of Death ,Humans ,Female ,Registries ,Research Article ,Aged ,Retrospective Studies - 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
- Published
- 2015