R Pérez-Alvarez1, P Brito-Zerón2, B Kostov3, C Feijoo-Massó4, G Fraile5, R Gómez-de-la-Torre6, B De-Escalante7, M López-Dupla8, A Alguacil9, J Chara-Cervantes10, M Pérez-Conesa11, J Rascón12, J S Garcia-Morillo13, P Perez-Guerrero14, E Fonseca-Aizpuru15, M Akasbi16, M Bonet17, J L Callejas18, L Pallarés12, M Ramos-Casals19. 1. Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Spain. 2. Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clinic, Barcelona, Spain; Systemic Autoimmune Diseases Unit, Hospital CIMA-Sanitas, Barcelona, Spain. 3. Primary Healthcare Transversal Research Group, IDIBAPS, Primary Care Center Les Corts, CAPSBE, Barcelona, Spain; Department of Statistics and Operational Research, Universitat Politècnica de Catalunya, Barcelona, Spain. 4. Department of Internal Medicine, Hospital Parc Tauli, Sabadell, Spain. 5. Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain. 6. Department of Internal Medicine, Hospital Universitario Central de Asturias (HUCA), Oviedo, Spain. 7. Department of Internal Medicine, Hospital Clínico, Zaragoza, Spain. 8. Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain. 9. Department of Internal Medicine, Hospital Virgen de la Salud, Toledo, Spain. 10. Department of Internal Medicine, Hospital Josep Trueta, Girona, Spain. 11. Department of Internal Medicine, Hospital Miguel Servet, Zaragoza, Spain. 12. Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca, Spain. 13. Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Spain. 14. Department of Internal Medicine, Hospital Puerta del Mar, Cadiz, Spain. 15. Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain. 16. Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain. 17. Department of Internal Medicine, Althaia, Xarxa Assistencial de Manresa, Manresa, Spain. 18. Department of Internal Medicine, Hospital San Cecilio, Granada, Spain. 19. Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clinic, Barcelona, Spain. Electronic address: mramos@clinic.ub.es.
Abstract
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.
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.
Authors: P Brito-Zerón; B Gracia-Tello; A Robles; A Alguacil; M Bonet; B De-Escalante; A Noblejas-Mosso; R Gómez-de-la-Torre; M Akasbi; M Pérez-de-Lis; R Pérez-Alvarez; M Ramos-Casals Journal: Viruses Date: 2021-05-27 Impact factor: 5.048