Pilar Brito-Zerón1, Roberto Pérez-Alvarez2, Carles Feijoo-Massó3, Borja Gracia-Tello4, Andres González-García5, Ricardo Gómez-de-la-Torre6, Ana Alguacil7, Miguel López-Dupla8, Angel Robles9, Salvador Garcia-Morillo10, Mariona Bonet11, Gracia Cruz-Caparrós12, Eva Fonseca-Aizpuru13, Miriam Akasbi14, Jose Luis Callejas15, Borja de Miguel-Campo16, Marta Pérez-de-Lis17, Manuel Ramos-Casals18. 1. Systemic Autoimmune Diseases Unit, Hospital CIMA-Sanitas, Barcelona, Spain. 2. Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Spain. 3. Department of Internal Medicine, Hospital Parc Tauli, Sabadell, Spain. 4. Department of Internal Medicine, Hospital Clínico, Zaragoza, 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 Virgen de la Salud, Toledo, Spain. 8. Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain. 9. Department of Internal Medicine, Hospital La Paz, Madrid, Spain. 10. Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Spain. 11. Department of Internal Medicine, Althaia, Xarxa Assistencial de Manresa, Manresa, Spain. 12. Department of Internal Medicine, Hospital de Poniente, Almería, Spain. 13. Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain. 14. Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain. 15. Department of Internal Medicine, Hospital San Cecilio, Granada, Spain. 16. Department of Internal Medicine, Hospital 12 de Octubre, Madrid, Spain. 17. Department of Anesthesiology, Complejo Hospitalario Universitario de A Coruña, A Coruña, Spain. 18. Department of Medicine, Universitat de Barcelona, Barcelona, Spain; Department of Autoimmune Diseases, ICMiD, Hospital Clinic, Barcelona, Spain. Electronic address: mramos@clinic.cat.
Abstract
OBJECTIVE: To analyze whether immune-mediated diseases (IMDs) occurs in sarcoidosis more commonly than expected in the general population, and how concomitant IMDs influence the clinical presentation of the disease. METHODS: We searched for coexisting IMDs in patients included in the SARCOGEAS-cohort, a multicenter nationwide database of consecutive patients diagnosed according to the ATS/ESC/WASOG criteria. Comparisons were made considering the presence or absence of IMD clustering, and odds ratios (OR) and their 95% confidence intervals (CI) were calculated as the ratio of observed cases of every IMD in the sarcoidosis cohort to the observed cases in the general population. RESULTS: Among 1737 patients with sarcoidosis, 283 (16%) patients presented at least one associated IMD. These patients were more commonly female (OR: 1.98, 95% CI: 1.49-2.62) and were diagnosed with sarcoidosis at an older age (49.6 vs. 47.5years, P<0.05). The frequency of IMDs in patients with sarcoidosis was nearly 2-fold higher than the frequency observed in the general population (OR: 1.64, 95% CI: 1.44-1.86). Significant associations were identified in 17 individual IMDs. In comparison with the general population, the IMDs with the strongest strength of association with sarcoidosis (OR>5) were common variable immunodeficiency (CVID) (OR: 431.8), familial Mediterranean fever (OR 33.9), primary biliary cholangitis (OR: 16.57), haemolytic anemia (OR: 12.17), autoimmune hepatitis (OR: 9.01), antiphospholipid syndrome (OR: 8.70), immune thrombocytopenia (OR: 8.43), Sjögren syndrome (OR: 6.98), systemic sclerosis (OR: 5.71), ankylosing spondylitis (OR: 5.49), IgA deficiency (OR: 5.07) and psoriatic arthritis (OR: 5.06). Sex-adjusted ORs were considerably higher than crude ORs for eosinophilic digestive disease in women, and for immune thrombocytopenia, systemic sclerosis and autoimmune hepatitis in men. CONCLUSION: We found coexisting IMDs in 1 out of 6 patients with sarcoidosis. The strongest associations were found for immunodeficiencies and some systemic, rheumatic, hepatic and hematological autoimmune diseases.
OBJECTIVE: To analyze whether immune-mediated diseases (IMDs) occurs in sarcoidosis more commonly than expected in the general population, and how concomitant IMDs influence the clinical presentation of the disease. METHODS: We searched for coexisting IMDs in patients included in the SARCOGEAS-cohort, a multicenter nationwide database of consecutive patients diagnosed according to the ATS/ESC/WASOG criteria. Comparisons were made considering the presence or absence of IMD clustering, and odds ratios (OR) and their 95% confidence intervals (CI) were calculated as the ratio of observed cases of every IMD in the sarcoidosis cohort to the observed cases in the general population. RESULTS: Among 1737 patients with sarcoidosis, 283 (16%) patients presented at least one associated IMD. These patients were more commonly female (OR: 1.98, 95% CI: 1.49-2.62) and were diagnosed with sarcoidosis at an older age (49.6 vs. 47.5years, P<0.05). The frequency of IMDs in patients with sarcoidosis was nearly 2-fold higher than the frequency observed in the general population (OR: 1.64, 95% CI: 1.44-1.86). Significant associations were identified in 17 individual IMDs. In comparison with the general population, the IMDs with the strongest strength of association with sarcoidosis (OR>5) were common variable immunodeficiency (CVID) (OR: 431.8), familial Mediterranean fever (OR 33.9), primary biliary cholangitis (OR: 16.57), haemolytic anemia (OR: 12.17), autoimmune hepatitis (OR: 9.01), antiphospholipid syndrome (OR: 8.70), immune thrombocytopenia (OR: 8.43), Sjögren syndrome (OR: 6.98), systemic sclerosis (OR: 5.71), ankylosing spondylitis (OR: 5.49), IgA deficiency (OR: 5.07) and psoriatic arthritis (OR: 5.06). Sex-adjusted ORs were considerably higher than crude ORs for eosinophilic digestive disease in women, and for immune thrombocytopenia, systemic sclerosis and autoimmune hepatitis in men. CONCLUSION: We found coexisting IMDs in 1 out of 6 patients with sarcoidosis. The strongest associations were found for immunodeficiencies and some systemic, rheumatic, hepatic and hematological autoimmune diseases.