1. First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study
- Author
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Mayka Freire, Juan José Ríos-Blanco, A.J. Chamorro, X Pla, D Colunga, Xavier Pla-Salas, B Marí, María Victoria Egurbide-Arberas, Melany Pestaña-Fernández, F J García Hernández, A Soto, M Pestaña, Gerard Espinosa, Luis Trapiella-Martínez, Ana Belén Madroñero-Vuelta, Vicent Fonollosa-Pla, P Segovia, L Sáez Comet, A. Guillén del Castillo, Dolores Colunga-Argüelles, A B Madroñero, Alfredo Guillén-del Castillo, M Ruiz Muñoz, María Victoria Egurbide, G Verdejo, Jose Antonio Todolí-Parra, C. Tolosa, V. Fonollosa, Carles Tolosa-Vilella, J A Vargas Hitos, Mónica Rodríguez-Carballeira, Manuel Ruiz-Muñoz, R González León, M Pérez Conesa, M Rubio Rivas, E Tarí, R Lorenzo, E Calvo, Norberto Ortego-Centeno, José Antonio Vargas-Hitos, Carmen Pilar Simeón-Aznar, María Jesús Castillo-Palma, Luis Sáez-Comet, M Freire, C.P. Simeon, L. Trapiella, J J Ríos Blanco, M.J. Castillo, A Marín, J A Todolí, M Rodríguez Carballeira, Pablo Segovia-Alonso, N Ortego-Centeno, Xavier Corbella, C Carbonell, Nerea Iniesta-Arandia, N Iniesta, X Corbella, Adela Marín-Ballvé, E Callejas Moraga, and Manuel Rubio-Rivas
- Subjects
Adult ,Male ,medicine.medical_specialty ,Prognostic factor ,Multivariate analysis ,Hypertension, Pulmonary ,Scleroderma Renal Crisis ,Arthritis ,030204 cardiovascular system & hematology ,Severity of Illness Index ,Scleroderma ,Raynaud ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,Internal medicine ,medicine ,Humans ,Registries ,Aged ,Retrospective Studies ,030203 arthritis & rheumatology ,Onset ,Scleroderma, Systemic ,business.industry ,Interstitial lung disease ,Raynaud Disease ,General Medicine ,Middle Aged ,Prognosis ,medicine.disease ,Arthralgia ,Systemic sclerosis ,Female ,Symptom Assessment ,Lung Diseases, Interstitial ,business ,Cohort study - Abstract
The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited. One thousand three hundred forty-two patients (83%) presented with RP as first symptom and 283 patients (17%) did not. Survival from first symptom in those patients with RP mode of onset was higher at any time than those with onset as non-Raynaud's phenomenon: 97 vs. 90% at 5 years, 93 vs. 82% at 10 years, 83 vs. 62% at 20 years, and 71 vs. 50% at 30 years (p < 0.001). In multivariate analysis, factors related to mortality were older age at onset, male gender, dcSSc subset, ILD, PAH, scleroderma renal crisis (SRC), heart involvement, and the mode of onset with non-Raynaud's phenomenon, especially in the form of puffy hands or pulmonary involvement. The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud's phenomenon may be considered of poor prognosis.
- Published
- 2017
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