OTOİMMÜN KARACİĞER HASTALARINDA İMMÜNOLOJİK DEĞERLENDİRME
AYAR, ŞEFİKA NUR
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Ayar, ŞN. Immunological Evaluation of Patients with Autoimmune Liver Diseases, Hacettepe University Faculty of Medicine, Thesis in Internal Medicine Department, Ankara, 2021. Objective: This study aimed to investigate primary immunodeficiency (PID) among patients with autoimmune liver disease (AILD). Materials and Methods: The diagnoses of the patients with AILD were reviewed according to the current guidelines, and these revised diagnoses for AILD were used for evaluation. A detailed immunological history was taken from the patients and laboratory values were obtained from electronic system records. All patients were screened for the PID together with immunologist. Those patients needing investigation for PID were referred to the immunology department, and were individually evaluated by further laboratory tests Results: There were 90 AILD patients who were followed up in the outpatient clinic (38 autoimmun hepatitis (AIH), 29 primary biliary cholangitis (PBC), 5 primary sclerosing cholangitis (PSC), and 18 variant syndromes (VS) were changed to 43 AIH, 32 PBC, 6 PSC, and 8 VS after re-evaluation, and AILD was excluded in 1 patient. In 7 of 89 patients included in this study, PID could not be excluded in the first evaluation, and it was planned to monitor them in terms of PID and complete evaluation after covid19 pandemic. Out of remaining 82 patients, 15 (18%) patients had diagnosis of PID; 3 combined immunodeficiency (CID), 4 common variable immunodeficiency (CVID), 4 partial IgA deficiency (PIgAD) and 4 selective IgM deficiency (SIgMD). PID was detected in 23% of AIH (9/39; 4 PIgAD, 3 SIgMD, 2 CVID); 9% of PBC (3/32; 2 CID, 1 CVID); 25% of PSC (1/4; 1 SIgMD), 29% of VS (2/7; 1 CID, 1 CVID) patients. It was observed that patients with PID had upper and lower respiratory tract infections more frequently than those without PID. No significant difference was observed in patients with and without PID in terms of treatment response, accompanying autoimmunity or malignancy, familial history for autoimmunity or malignancy, and parental consanguinity. Conclusion: Although PIDs are rare diseases, the frequency of PID was higher in AILD compared to the general population. Further researches with larger AILD patient groups are needed in order to evaluate prognostic impacts of PID on patients with AILD.