Portal de Periódicos da CAPES

Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients

2019; Elsevier BV; Volume: 69; Linguagem: Inglês

10.1016/j.ejim.2019.08.025

1879-0828

R. Pérez-Álvarez, Pilar Brito‐Zerón, Belchin Kostov, Carlos Feijoo-Massó, G. Fraile, Ricardo Gómez-de-la-Torre, B. De-Escalante, Miguel López-Dupla, Ana Alguacil, J. Chara-Cervantes, Mercedes Pérez-Conesa, Javier Rascón, José Salvador García Morillo, P. Pérez‐Guerrero, Eva Fonseca‐Aizpuru, Miriam Akasbi, Mariona Bonet, José Luis Callejas‐Rubio, L Pallarés, Manuel Ramos‐Casals, R. Pérez-Álvarez, Pilar Brito‐Zerón, Belchin Kostov, Carlos Feijoo-Massó, G. Fraile, Ricardo Gómez-de-la-Torre, B. De-Escalante, Miguel López-Dupla, Ana Alguacil, J. Chara-Cervantes, Mercedes Pérez-Conesa, Javier Rascón, José Salvador García Morillo, P. Pérez‐Guerrero, Eva Fonseca‐Aizpuru, Miriam Akasbi, Mariona Bonet, José Luis Callejas‐Rubio, G. De-la-Red, E. Calvo, Cristina Soler, E. Peral-Gutiérrez, J.F. Gómez-Cerezo, Gracia Cruz-Caparrós, Sara Rodríguez Fernández, Blanca Pinilla, A Gato, Antonio Javier Chamorro Fernández, C. Morcillo, Ángel Robles‐Marhuenda, I Ojeda, M.J. Vives, Borja de Miguel-Campo, M. Penadés, M. De-Vicente, Xavier Bosch, M. Pérez-de-Lis, Andrés González García, C. Yllera, Borja Gracia-Tello, Alexandre Pérez‐González, M. Pedrosa-Aragón, Carles Tolosa, Antoni Sisó‐Almirall, L Pallarés, Manuel Ramos‐Casals,

Autoimmune and Inflammatory Disorders

Background To analyze the association between Scadding radiological stages of sarcoidosis at diagnosis and the disease phenotype (epidemiology, clinical presentation and extrathoracic involvement) in one of the largest cohorts of patients with sarcoidosis reported from southern Europe. Methods The SARCOGEAS-Study Group includes a multicenter database of consecutive patients diagnosed with sarcoidosis according to the WASOG 1999 criteria. Extrathoracic disease at diagnosis was defined according to the 2014 instrument and the clusters proposed by Schupp et al. Results We analyzed 1230 patients (712 female, mean age 47 yrs.) who showed the following Scadding radiologic stages at diagnosis: stage 0 (n = 98), stage I (n = 395), stage II (n = 500), stage III (n = 195) and stage IV (n = 42). Women were overrepresented in patients presenting with extrathoracic/extrapulmonary disease, while the diagnosis was made at younger ages in patients presenting with BHL, and at older ages in those presenting with pulmonary fibrosis (q values <0.05). Multivariable adjusted analysis showed that patients presenting with pulmonary involvement (especially those with stages II and III) had a lower frequency of concomitant systemic involvement in some specific extrathoracic clusters (cutaneous-adenopathic/musculoskeletal, ENT and neuro-ocular/OCCC) but a higher frequency for others (hepatosplenic), in comparison with patients with extrapulmonary involvement (stages 0 and I). The presence of either BHL or fibrotic lesions did not influence the systemic phenotype of patients with pulmonary involvement. Conclusions The key determinant associated with a differentiated systemic phenotype of sarcoidosis at diagnosis was interstitial pulmonary involvement rather than the individual Scadding radiological stage.