Abstract
Purpose: PIDs associates with autoimmune diseases include autoimmune liver diseases (AILD); however, the frequency of PIDs among patients with AILD is unknown. This study aimed to evaluate the strength of the association between AILD and PIDs. Methods: We conducted this single-center, cross-sectional, and descriptive study in a tertiary hospital. We evaluated eighty-two patients with AILD (39 autoimmune hepatitis (AIH), 32 with primary biliary cholangitis (PBC), seven with variant syndromes (VS), and four with primary sclerosing cholangitis (PSC) for the presence of PIDs. We obtained a detailed history of infections, comorbidities, family history, and laboratory data from the files. All patients were evaluated in the immunology department for further examination, and PID diagnoses were made according to ESID (The European Society for Immunodeficiencies) criteria. Results: Out of 82 patients with AILD, PIDs were diagnosed in 18% (15 patients); there were four patients with common variable immunodeficiency (CVID), four with partial IgA deficiency (PIgAD), four with selective IgM deficiency (SIgMD), and three with combined immunodeficiency (CID). PIDs were present in 29% of patients with VS, 25 % of patients with PSC, 23% of patients with AIH, and 9% of patients with PBC. Conclusion: Although PIDs are rare diseases in the general population, they have a strong association with AILD and were detected in one-fifth of the patients. Further research with larger patient groups is needed to evaluate the diagnostic and prognostic impacts of PIDs on AILD.
264 Screening for antibodies, associated with autoimmune liver diseases in children with celiac disease
