title: Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.
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.
authors.affiliation: From the Department of Internal Medicine, Hospital Valld'Hebron (CPS-A, VF-P, MV-T); Department of Internal Medicine, Hospital Parc Taulí, Sabadell (CT-V); Department of Autoimmune Diseases, Hospital Clinic (GE-G, MR-C); Laboratori of Computacional Medicine, Bioestatistics Unit, Universitat Autònoma de Barcelona, Bellaterra, Barcelona (MC-G); Unit of Connective Tissue Diseases, Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla (FJG-H, MJC-P, JS-R); Unit of Autoimmune Systemic Diseases, Department of Internal Medicine, Hospital Clínico San Cecilio, Granada (JLC-R, NO-C); Department of Internal Medicine, Hospital de Cruces, Galdakano, Bilbao (MVE-A); Department of Internal Medicine, Hospital de Cabueñes, Gijón (LT-M); Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo (LC-M); Department of Internal Medicine, Hospital Miguel Servet, Zaragoza (LS-C, JV-M); Department of Internal Medicine, Hospital Carlos Haya, Málaga (MTC-G, ER-G); Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca (EME-M, LP-F); Department of Internal Medicine, Hospital Virgen de las Nieves, Granada (NN-N, JAV-H); Department of Internal Medicine, Hospital San Agustín, Avilés (RGT); Department of Internal Medicine, Hospital La Fe, Valencia (GS-C); and Department of Internal Medicine, Hospital La Paz, Madrid (JJR-B), Systemic Autoimmune Diseases Group (GEAS), Spanish Scleroderma Study Group (SSSG), Spanish Society of Internal Medicine, Spain.
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authors.initials: CP
authors.lastname: Simeón-Aznar
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authors.lastname: Ramos-Casals
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authors.lastname: Castillo-Palma
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authors.lastname: Sánchez-Román
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authors.initials: JL
authors.lastname: Callejas-Rubio
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authors.lastname: Ortego-Centeno
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authors.initials: MV
authors.lastname: Egurbide-Arberas
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authors.lastname: Trapiellla-Martínez
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authors.lastname: Caminal-Montero
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authors.lastname: Sáez-Comet
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authors.lastname: Camps-García
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authors.lastname: de Ramón-Garrido
authors.forename: E M
authors.initials: EM
authors.lastname: Esteban-Marcos
authors.forename: L
authors.initials: L
authors.lastname: Pallarés-Ferreres
authors.forename: N
authors.initials: N
authors.lastname: Navarrete-Navarrete
authors.forename: J A
authors.initials: JA
authors.lastname: Vargas-Hitos
authors.forename: R Gómez de la
authors.initials: RG
authors.lastname: Torre
authors.forename: G
authors.initials: G
authors.lastname: Salvador-Cervello
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authors.initials: JJ
authors.lastname: Rios-Blanco
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authors.initials: M
authors.lastname: Vilardell-Tarrés
mesh_terms: D000328:Adult; D000368:Aged; D002423:Cause of Death; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000401:mortality*; D013030:Spain / Q000453:epidemiology