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First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study.
Clin Rheumatol. 2018 Apr; 37(4):999-1009.CR

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.

Authors+Show Affiliations

Department of Internal Medicine, Hospital Universitario de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain. mrubio@bellvitgehospital.cat.Department of Internal Medicine, Hospital Universitario de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain. Faculty of Medicine and Health Sciences, Universitat Internacional de Catalunya, Barcelona, Spain.Department of Internal Medicine, Hospital Universitario de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain.Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona, Spain.Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain.Department of Internal Medicine, Hospital de Cabueñes, Gijón, Asturias, Spain.Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatología, Hospital Clínic, Barcelona, Spain.Collagenosis and Pulmonary Hypertension Unit, Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Sevilla, Spain.Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.Department of Internal Medicine, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain.Department of Internal Medicine, Hospital Universitario San Cecilio, Granada, Spain.Thrombosis and Vasculitis Unit, Department of Internal Medicine, Complexo Hospitalario Universitario de Vigo, Vigo, Spain.Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain.Department of Internal Medicine, Hospital Universitario La Paz, Madrid, Spain.Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain.Department of Internal Medicine, Hospital Universitari Mútua Terrassa, Barcelona, Spain.Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain.Department of Internal Medicine, Consorci Hospitalari de Vic, Barcelona, Spain.Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain.Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Madrid, Spain.Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29214548

Citation

Rubio-Rivas, Manuel, et al. "First Clinical Symptom as a Prognostic Factor in Systemic Sclerosis: Results of a Retrospective Nationwide Cohort Study." Clinical Rheumatology, vol. 37, no. 4, 2018, pp. 999-1009.
Rubio-Rivas M, Corbella X, Pestaña-Fernández M, et al. First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study. Clin Rheumatol. 2018;37(4):999-1009.
Rubio-Rivas, M., Corbella, X., Pestaña-Fernández, M., Tolosa-Vilella, C., Guillen-Del Castillo, A., Colunga-Argüelles, D., Trapiella-Martínez, L., Iniesta-Arandia, N., Castillo-Palma, M. J., Sáez-Comet, L., Egurbide-Arberas, M. V., Ortego-Centeno, N., Freire, M., Vargas-Hitos, J. A., Ríos-Blanco, J. J., Todolí-Parra, J. A., Rodríguez-Carballeira, M., Marín-Ballvé, A., Segovia-Alonso, P., ... Verdejo, G. (2018). First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study. Clinical Rheumatology, 37(4), 999-1009. https://doi.org/10.1007/s10067-017-3936-7
Rubio-Rivas M, et al. First Clinical Symptom as a Prognostic Factor in Systemic Sclerosis: Results of a Retrospective Nationwide Cohort Study. Clin Rheumatol. 2018;37(4):999-1009. PubMed PMID: 29214548.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study. AU - Rubio-Rivas,Manuel, AU - Corbella,Xavier, AU - Pestaña-Fernández,Melany, AU - Tolosa-Vilella,Carles, AU - Guillen-Del Castillo,Alfredo, AU - Colunga-Argüelles,Dolores, AU - Trapiella-Martínez,Luis, AU - Iniesta-Arandia,Nerea, AU - Castillo-Palma,María Jesús, AU - Sáez-Comet,Luis, AU - Egurbide-Arberas,María Victoria, AU - Ortego-Centeno,Norberto, AU - Freire,Mayka, AU - Vargas-Hitos,Jose Antonio, AU - Ríos-Blanco,Juan José, AU - Todolí-Parra,Jose Antonio, AU - Rodríguez-Carballeira,Mónica, AU - Marín-Ballvé,Adela, AU - Segovia-Alonso,Pablo, AU - Pla-Salas,Xavier, AU - Madroñero-Vuelta,Ana Belén, AU - Ruiz-Muñoz,Manuel, AU - Fonollosa-Pla,Vicent, AU - Simeón-Aznar,Carmen Pilar, AU - ,, AU - Callejas Moraga,E, AU - Calvo,E, AU - Carbonell,C, AU - Castillo,M J, AU - Chamorro,A J, AU - Colunga,D, AU - Corbella,X, AU - Egurbide,M V, AU - Espinosa,G, AU - Fonollosa,V, AU - Freire,M, AU - García Hernández,F J, AU - González León,R, AU - Guillén Del Castillo,A, AU - Iniesta,N, AU - Lorenzo,R, AU - Madroñero,A B, AU - Marí,B, AU - Marín,A, AU - Ortego-Centeno,N, AU - Pérez Conesa,M, AU - Pestaña,M, AU - Pla,X, AU - Ríos Blanco,J J, AU - Rodríguez Carballeira,M, AU - Rubio Rivas,M, AU - Ruiz Muñoz,M, AU - Sáez Comet,L, AU - Segovia,P, AU - Simeón,C P, AU - Soto,A, AU - Tarí,E, AU - Todolí,J A, AU - Tolosa,C, AU - Trapiella,L, AU - Vargas Hitos,J A, AU - Verdejo,G, Y1 - 2017/12/07/ PY - 2017/07/14/received PY - 2017/11/27/accepted PY - 2017/11/07/revised PY - 2017/12/8/pubmed PY - 2018/9/8/medline PY - 2017/12/8/entrez KW - Onset KW - Prognosis KW - Raynaud KW - Scleroderma KW - Systemic sclerosis SP - 999 EP - 1009 JF - Clinical rheumatology JO - Clin. Rheumatol. VL - 37 IS - 4 N2 - 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. SN - 1434-9949 UR - https://www.unboundmedicine.com/medline/citation/29214548/First_clinical_symptom_as_a_prognostic_factor_in_systemic_sclerosis:_results_of_a_retrospective_nationwide_cohort_study_ L2 - https://dx.doi.org/10.1007/s10067-017-3936-7 DB - PRIME DP - Unbound Medicine ER -