Coexistence of immune-mediated diseases in sarcoidosis. Frequency and clinical significance in 1737 patients - 18/11/21
The SarcoGEAS-SEMI Registry
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Highlights |
• | 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. |
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.
Il testo completo di questo articolo è disponibile in PDF.Keywords : Sarcoidosis, Autoimmune diseases, Immunodeficiencies, Sjögren syndrome, Systemic sclerosis, Antiphospholipid syndrome, Primary biliary cholangitis, Autoimmune hepatitis, Cytopenias, Arthritis
Mappa
Vol 88 - N° 6
Articolo 105236- Dicembre 2021 Ritorno al numero
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