%0 Journal Article
%A Simeón-Aznar, C P
%A Fonollosa-Plá, V
%A Tolosa-Vilella, Carles
%A Espinosa-Garriga, G
%A Campillo-Grau, M
%A Ramos-Casals, M
%A García-Hernández, F J
%A Castillo-Palma, M J
%A Sánchez-Román, J
%A Callejas-Rubio, J L
%A Ortego-Centeno, N
%A Egurbide-Arberas, M V
%A Trapiellla-Martínez, L
%A Caminal-Montero, L
%A Sáez-Comet, L
%A Velilla-Marco, J
%A Camps-García, M T
%A de Ramón-Garrido, E
%A Esteban-Marcos, E M
%A Pallarés-Ferreres, L
%A Navarrete-Navarrete, N
%A Vargas-Hitos, J A
%A Gómez de la Torre, R
%A Salvador-Cervello, G
%A Rios-Blanco, J J
%A Vilardell-Tarrés, M
%T Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.
%D 2015
%@ 0025-7974
%U http://hdl.handle.net/10668/2607
%X 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
%K Síndrome CREST
%K Causas de muerte
%K Progresión de la enfermedad
%K Humanos
%K Hipertensión pulmonar
%K Enfermedades pulmonares Intersticiales
%K Masculino
%K Análisis multivariante
%K Prevalencia
%K Pronóstico
%K Modelos de riesgos proporcionales
%K Factores de riesgo
%K Esclerodermia difusa
%K Esclerodermia limitada
%K Encuestas y cuestionarios
%K Tasa de supervivencia
%K Úlcera
%K España
%~