The association of primary immunodeficiency's with autoimmune liver diseases is not rare

Autoimmune Liver Disorders (AILD) are PIDs associated with autoimmune diseases; however, it is uncertain how common PIDs are in AILD patients. The purpose of this study was to assess how strongly AILD and PIDs are related. In a tertiary hospital, we carried out this single-center, cross-sectional, and descriptive study. Eighty-two AILD patients were examined for the presence of PIDs, including 39 with Autoimmune Hepatitis (AIH), 32 with Primary Biliary Cholangitis (PBC), seven with Variant Syndromes (VS), and four with Primary Sclerosing Cholangitis (PSC). The files provided us with a thorough history of illnesses, comorbidities, family history, and laboratory information. The immunology division reviewed each patient for further testing, and PID diagnoses were established in accordance with ESID (The European Society for Immunodeficiency’s) standards. PIDs were discovered in 15% (82/82 patients) of patients with AILD, including 15 patients with SIgMD, 4 patients with partial IgA deficiency (PIgAD), 4 patients with Selective IgM Deficiency (SIgMD), and 3 patients with mixed immunodeficiency (CID). PIDs were found in 29% of VS patients, 25% of PSC patients, 23% of AIH patients, and 9% of PBC patients.