RT Journal Article
T1 Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.
A1 Simeón-Aznar, C P
A1 Fonollosa-Plá, V
A1 Tolosa-Vilella, Carles
A1 Espinosa-Garriga, G
A1 Campillo-Grau, M
A1 Ramos-Casals, M
A1 García-Hernández, F J
A1 Castillo-Palma, M J
A1 Sánchez-Román, J
A1 Callejas-Rubio, J L
A1 Ortego-Centeno, N
A1 Egurbide-Arberas, M V
A1 Trapiellla-Martínez, L
A1 Caminal-Montero, L
A1 Sáez-Comet, L
A1 Velilla-Marco, J
A1 Camps-García, M T
A1 de Ramón-Garrido, E
A1 Esteban-Marcos, E M
A1 Pallarés-Ferreres, L
A1 Navarrete-Navarrete, N
A1 Vargas-Hitos, J A
A1 Gómez de la Torre, R
A1 Salvador-Cervello, G
A1 Rios-Blanco, J J
A1 Vilardell-Tarrés, M
K1 Síndrome CREST
K1 Causas de muerte
K1 Progresión de la enfermedad
K1 Humanos
K1 Hipertensión pulmonar
K1 Enfermedades pulmonares Intersticiales
K1 Masculino
K1 Análisis multivariante
K1 Prevalencia
K1 Pronóstico
K1 Modelos de riesgos proporcionales
K1 Factores de riesgo
K1 Esclerodermia difusa
K1 Esclerodermia limitada
K1 Encuestas y cuestionarios
K1 Tasa de supervivencia
K1 Úlcera
K1 España
AB 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
PB Lippincott, Williams & Wilkins
SN 0025-7974
YR 2015
FD 2015-10-30
LK http://hdl.handle.net/10668/2607
UL http://hdl.handle.net/10668/2607
LA en
NO Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, Espinosa-Garriga G, Campillo-Grau M, Ramos-Casals M, et al. Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death. Medicine (Baltimore). 2015                         ; 94(43):e1728
DS RISalud
RD Apr 11, 2025