TY - JOUR
T1 - Registry of the Spanish network for systemic sclerosis: Survival, prognostic factors, and causes of death
AU - Simeón-Aznar, C. P.
AU - Fonollosa-Plá, V.
AU - Tolosa-Vilella, Carles
AU - Espinosa-Garriga, G.
AU - Campillo-Grau, M.
AU - Ramos-Casals, M.
AU - García-Hernández, F. J.
AU - Castillo-Palma, M. J.
AU - Sánchez-Román, J.
AU - Callejas-Rubio, J. L.
AU - Ortego-Centeno, N.
AU - Egurbide-Arberas, M. V.
AU - Trapiellla-Martínez, L.
AU - Caminal-Montero, L.
AU - Sáez-Comet, L.
AU - Velilla-Marco, J.
AU - Camps-García, M. T.
AU - De Ramón-Garrido, E.
AU - Esteban-Marcos, E. M.
AU - Pallarés-Ferreres, L.
AU - Navarrete-Navarrete, N.
AU - Vargas-Hitos, J. A.
AU - De La Torre, R. Gómez
AU - Salvador-Cervello, G.
AU - Rios-Blanco, J. J.
AU - Vilardell-Tarrés, M.
PY - 2015/1/1
Y1 - 2015/1/1
N2 - Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. 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 proportionalhazards 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.
AB - Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. 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 proportionalhazards 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.
U2 - 10.1097/MD.0000000000001728
DO - 10.1097/MD.0000000000001728
M3 - Article
VL - 94
IS - 43
M1 - e1728
ER -