Çocukluk Çağında Otoimmün Hepatit Hastalığı (82 Hastanın Klinik, Laboratuvar Ve Histopatolojik Bulgularının Değerlendirilmesi)

Abstract

Dölek Güvenkaya E., Autoimmune Hepatitis in Childhood (Evaluation of Clinical, Laboratory, and Histopathological Findings in 82 Patients), Hacettepe University Faculty of Medicine, Department of Pediatrics, Subspecialty Thesis in Ankara, 2023. Autoimmune hepatitis (AIH) is an immune-mediated liver disease diagnosed based on elevated transaminases, the presence of autoantibodies serologically, high IgG levels, and inflammatory liver histology after excluding other known causes of liver disease. In this study, 82 patients diagnosed with AIH and followed by the Hacettepe University Faculty of Medicine, Department of Pediatric Gastroenterology between January 1, 1994, and January 1, 2022, were retrospectively evaluated. Of the patients in the study group, 57.3% were female, and 42.7% were male. 63.4% had Type 1 AIH, 23.2% had Type 2 AIH, and 13.4% had seronegative AIH. The average age at diagnosis was 9.69±3.75 years for Type 1 AIH, 9.05±4.88 years for Type 2 AIH, and 10.17±4.42 years for seronegative AIH. The most common complaints at presentation were jaundice (43.9%), fatigue (39%), and abdominal pain (35.4%). According to the onset of the disease, 39.1% were similar to acute viral hepatitis, 31.7% were incidental, 14.6% had an insidious onset, 13.4% were diagnosed with cirrhosis, and 1.2% were diagnosed with acute liver failure. ANA positivity was found in 88.5% of Type 1 AIH patients, 25% had ASMA positivity, and 17.3% had both ANA and ASMA positivity. In Type 2 AIH patients, 84.2% had anti-LKM-1 positivity, 21% had anti-LC-1 positivity, and 5.3% had both anti-LKM-1 and anti-LC-1 positivity. Histopathological findings included lymphoplasmacytic cell infiltration in 95.1% of patients, interface hepatitis in 92.7%, bridging necrosis in 67.9%, and rosette formation in 28.4%. 51.9% of patients received a definite diagnosis of AIH, while 48.1% were probable AIH. All patients were treated with prednisolone at a dose of 2 mg/kg/day. Treatment consisted of prednisolone only in 12.2% of patients, prednisolone with simultaneous azathioprine in 14.6%, and azathioprine after prednisolone in 73.2%. 4.9% of patients receiving prednisolone and azathioprine also required mycophenolate mofetil (MMF) treatment. The remission status of patients is considered as follows: normalization of transaminase levels is termed "biochemical remission", normalization of both transaminase and IgG levels is termed "complete biochemical remission", and normalization of transaminase and IgG levels along with either negativity or significantly low titers of autoantibodies, accompanied by histological improvement of liver inflammation, is considered "complete clinical remission".61 patients achieved biochemical remission, with 60.6% having Type 1 AIH, 26.2% having Type 2 AIH, and 13.2% having seronegative AIH. 54 patients achieved complete biochemical remission, with 57.4% having Type 1 AIH, 27.8% having Type 2 AIH, and 14.8% having seronegative AIH. Among 10 patients who achieved complete clinical remission and discontinued treatment, 50% had Type 1 AIH, 20% had Type 2 AIH, and 30% had seronegative AIH. Complete biochemical remission was more commonly observed in patients without initial complaints (93.8%, p=0.02). Biochemical remission was statistically significantly higher in patients diagnosed with clinical symptoms similar to acute viral hepatitis (p=0.048), complete biochemical remission was higher in patients diagnosed incidentally (p=0.028), and complete clinical remission was lower in patients with insidious onset (p=0.023). Patients who achieved biochemical remission had a lower AIH diagnostic score than those who did not, and patients who achieved complete biochemical remission had a lower AIH diagnostic score than those who did not (p<0.05). At the last follow-up, 51.3% of patients had discontinued prednisolone treatment, and the prednisolone dose in 33.7% of patients was 5 mg/day as maintenance. Of the 72 patients started on azathioprine, treatment was discontinued in 8 patients due to remission and in 4 patients due to side effects. There was a negative correlation (r=-0.275) between the duration of prednisolone treatment and height standard deviation (SD) change, and a positive correlation (r=0.351) between the duration of prednisolone treatment and body mass index (BMI) SD change. The mortality rate was 2.4%. Establishing national databases and increasing awareness of AIH among healthcare professionals in our country can positively impact the treatment of the disease.