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

•Coexisting immune-mediated diseases (IMDs) were identified in 1 out of 6 patients with sarcoidosis.•The frequency of immune-mediated disease (IMDs) in our patients with sarcoidosis was 1.64-fold higher than that reported in general population.•Women with sarcoidosis have a two-fold higher frequency of concomitant IMDs.•We identify 5 key IMDs strongly associated with sarcoidosis (primary immunodeficiencies, systemic autoimmune diseases, inflammatory arthritis, autoimmune liver diseases and autoimmune cytopenias).•Search for coexisting IMDs in patients with sarcoidosis presenting with features considered out of the clinical extrathoracic scenario typical of sarcoidosis.•There was a wide variety of both systemic and organ-specific diseases coexisting with sarcoidosis. 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, P5) 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 considera