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Title: Registry of the Spanish network for systemic sclerosis: survival, prognostic factors, and causes of death
Author: Simeón Aznar, Carmen Pilar
Fonollosa Pla, Vicent
Tolosa Vilella, Carles
Espinosa Garriga, Gerard
Campillo Grau, M.
Ramos Casals, Manuel
García Hernández, F.J.
Castillo Palma, María Jesús
Sánchez Román, J.
Callejas Rubio, José Luis
Ortego Centeno, Norberto
Egurbide Arberas, María Victoria
Trapiellla Martínez, Luis
Caminal Montero, L.
Sáez Comet, Luis
Velilla Marco, J.
Camps García, María Teresa
Ramón Garrido, E . de
Esteban Marcos, E.M.
Pallarés Ferreres, L.
Navarrete Navarrete, N.
Vargas Hitos, José A.
Gómez de la Torre, R.
Salvador Cervello, G.
Ríos Blanco, Juan José
Vilardell Tarrés, M.
Spanish Scleroderma Study Group (SSSG)
Autoimmune Diseases Study Group (GEAS)
Spanish Society of Internal Medicine (SEMI)
Keywords: Esclerodèrmia
Malalties autoimmunitàries
Scleroderma (Disease)
Autoimmune diseases
Issue Date: Oct-2015
Publisher: Lippincott, Williams & Wilkins. Wolters Kluwer Health
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.
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It is part of: Medicine, 2015, vol. 94, num. 43, p. e1728
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ISSN: 0025-7974
Appears in Collections:Articles publicats en revistes (Medicina)

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