Very Early and Early Systemic Sclerosis in the Spanish Scleroderma Registry (RESCLE) Cohort

Publication date: Available online 28 May 2017
Source:Autoimmunity Reviews
Author(s): Luis Trapiella Martínez, José Bernardino Díaz López, Luis Caminal Montero, Carles Tolosa Vilella, Alfredo Guillén del Castillo, Dolores Colunga Argüelles, Manuel Rubio Rivas, 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, Antonio Javier Chamorro Fernández, Xavier Pla Salas, Ana Belén Madroñero Vuelta, Manuel Ruiz Muñoz, Vicent Fonollosa Pla, Carmen Pilar Simeón Aznar
ObjectivesAccording to the existence of subclinical organ involvement pre-scleroderma should be divided into two subsets: very early and early disease. Pre-scleroderma patients included in the Spanish Scleroderma Registry (RESCLE) Cohort were reclassified into subsets. Differences were evaluated and the risk of progression to definite systemic sclerosis was estimated.MethodsThe characteristics of very early and early SSc patients were compared. A logistic regression model was used to determine the risk factors of progression.Results1632 patients were included, 36 (2.2%) in the very early subset and 111 (6.8%) in the early subset. There were no differences in sex, age at disease onset, duration of Raynaud's phenomenon, antinuclear antibodies or capillaroscopic findings. Three (8.3%) very early SSc patients evolved to definite SSc, 2 (5.6%) of them meeting the ACR/EULAR 2013 criteria, unlike 31 (28%) early SSc patients, 20 (24%) of them meeting the criteria (p=0.034). Digestive involvement was an independent risk factor of progression (OR 17; 95% CI, 6.1–47.2).ConclusionsThe classification of early forms of scleroderma identifies patients with different prognostic risk of progression. The evolution to definite SSc is more frequent in early than in very early SSc patients. Digestive involvement is a risk factor of progression. An active assessment of organ damage in preclinical stages allows a correct classification and risk stratification, with implications for monitoring and treatment.



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