Simeón-Aznar, C PFonollosa-Plá, VTolosa-Vilella, CarlesEspinosa-Garriga, GCampillo-Grau, MRamos-Casals, MGarcía-Hernández, F JCastillo-Palma, M JSánchez-Román, JCallejas-Rubio, J LOrtego-Centeno, NEgurbide-Arberas, M VTrapiellla-Martínez, LCaminal-Montero, LSáez-Comet, LVelilla-Marco, JCamps-García, M Tde Ramón-Garrido, EEsteban-Marcos, E MPallarés-Ferreres, LNavarrete-Navarrete, NVargas-Hitos, J AGómez de la Torre, RSalvador-Cervello, GRios-Blanco, J JVilardell-Tarrés, M2017-04-062017-04-062015-10-30Simeón-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):e17280025-7974http://hdl.handle.net/10668/2607Systemic 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 factorsenSíndrome CRESTCausas de muerteProgresión de la enfermedadHumanosHipertensión pulmonarEnfermedades pulmonares IntersticialesMasculinoAnálisis multivariantePrevalenciaPronósticoModelos de riesgos proporcionalesFactores de riesgoEsclerodermia difusaEsclerodermia limitadaEncuestas y cuestionariosTasa de supervivenciaÚlceraEspañaMedical Subject Headings::Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Limited::CREST SyndromeMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Cause of DeathMedical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Disease ProgressionMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Diseases::Respiratory Tract Diseases::Lung Diseases::Hypertension, PulmonaryMedical Subject Headings::Diseases::Respiratory Tract Diseases::Lung Diseases::Lung Diseases, InterstitialMedical Subject Headings::Check Tags::MaleMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Analysis of Variance::Multivariate AnalysisMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::PrevalenceMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::PrognosisMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Models, Statistical::Proportional Hazards ModelsMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Probability::Risk::Risk FactorsMedical Subject Headings::Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, DiffuseMedical Subject Headings::Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, LimitedMedical Subject Headings::Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, SystemicMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::QuestionnairesMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Survival RateMedical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::UlcerMedical Subject Headings::Geographical Locations::Geographic Locations::Europe::Spainresearch article26512564open access10.1097/MD.00000000000017281536-5964PMC4985378