Title |
First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study
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Published in |
Clinical Rheumatology, December 2017
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DOI | 10.1007/s10067-017-3936-7 |
Pubmed ID | |
Authors |
Manuel Rubio-Rivas, Xavier Corbella, Melany Pestaña-Fernández, Carles Tolosa-Vilella, Alfredo Guillen-del Castillo, Dolores Colunga-Argüelles, Luis Trapiella-Martínez, Nerea Iniesta-Arandia, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan José Ríos-Blanco, Jose Antonio Todolí-Parra, Mónica Rodríguez-Carballeira, Adela Marín-Ballvé, Pablo Segovia-Alonso, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñoz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar, on behalf of RESCLE investigators, Autoimmune Diseases Study Group (GEAS), E Callejas Moraga, E. Calvo, C. Carbonell, M. J. Castillo, A. J. Chamorro, D. Colunga, X. Corbella, M. V. Egurbide, G. Espinosa, V. Fonollosa, M. Freire, F. J. García Hernández, R. González León, A. Guillén del Castillo, N. Iniesta, R. Lorenzo, A. B. Madroñero, B. Marí, A. Marín, N. Ortego-Centeno, M. Pérez Conesa, M. Pestaña, X. Pla, J. J. Ríos Blanco, M. Rodríguez Carballeira, M. Rubio Rivas, M. Ruiz Muñoz, L. Sáez Comet, P. Segovia, C. P. Simeón, A. Soto, E. Tarí, J. A. Todolí, C. Tolosa, L. Trapiella, J. A. Vargas Hitos, G. Verdejo |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 3 | 20% |
United Kingdom | 2 | 13% |
Spain | 2 | 13% |
Dominican Republic | 2 | 13% |
Canada | 1 | 7% |
Colombia | 1 | 7% |
Unknown | 4 | 27% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 10 | 67% |
Practitioners (doctors, other healthcare professionals) | 4 | 27% |
Scientists | 1 | 7% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 64 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Other | 11 | 17% |
Professor > Associate Professor | 8 | 13% |
Student > Bachelor | 6 | 9% |
Researcher | 6 | 9% |
Student > Postgraduate | 5 | 8% |
Other | 12 | 19% |
Unknown | 16 | 25% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 33 | 52% |
Materials Science | 2 | 3% |
Agricultural and Biological Sciences | 2 | 3% |
Computer Science | 1 | 2% |
Immunology and Microbiology | 1 | 2% |
Other | 5 | 8% |
Unknown | 20 | 31% |