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Coexistence of immune-mediated diseases in sarcoidosis. Frequency and clinical significance in 1737 patients

2021; Elsevier BV; Volume: 88; Issue: 6 Linguagem: Inglês

10.1016/j.jbspin.2021.105236

1778-7254

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

IgG4-Related and Inflammatory Diseases

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.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.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 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.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.