الفهرس 
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Abstract
Summary
Autoimmune hepatitis is a progressive inflammatory liver disorder of unknown etiology. It is classified into two main types according to the circulating autoantibodies. It has a very wide spectrum of clinical presentations ranging from being asymptomatic to an acute severe fulminant disease. Other associated autoimmune diseases are very common including AIT. The aim of this study was to screen children with AIH for associated AIT.
This cross-sectional study included 58 children presented to the Pediatric Hepatology Unit at Beni-Suef University Hospital and Cairo University Pediatric Hospital, Egypt during the period from May 2019 to May 2021 with established diagnosis of AIH. The clinical, laboratory data, therapy and outcome were retrieved from the patients’ files and from patients. A 5 ml blood sample was withdrawn from a peripheral vein under aseptic condition on a plain tube. Serum was analyzed to detect free T3, free T4, TSH, anti-thyroid peroxidase and anti-thyroglobulin to screen for associated AIT.
The study included 58 AIH patients. Female: male ratio was 2.2:1. Mean ± SD for their ages was 11.3 ± 4.5 years. The mean ± SD age at disease onset was 6.8 ± 3.9 years. Thirty-one patients (53.4%) presented with acute hepatitic illness, meanwhile 11 patients (19%) presented with acute liver cell failure, 8 patients (13.8%) with insidious onset and another 8 patients (13.8) with complications of chronic liver disease in the form of: splenomegaly, portal HTN, hematemesis or melena.
Jaundice was the main presenting symptom in 47 patients (81%). Transaminases were elevated in all patients on initial presentation. IgG was elevated in 55 patients (94.8%) with a mean value of 2832 ± 1249 g/L. Anti-smooth muscle antibody (ASMA) was the most commonly detected autoantibody (in 77.6% of the patients). Forty patients (69%) had type 1 AIH, while fifteen (25.9%) had type 2 AIH and three patients (5.1%) was autoantibody negative AIH. By liver biopsy, hepatic architecture was distorted in 81.1% of the patients and piecemeal necrosis was observed in 83.8%.
The initial treatment was oral prednisolone at a dose 2 mg/ kg/ day. Azathioprine was added to prednisolone in the treatment of 50% of the patients. There was a statistically significant improvement in the level of bilirubin, transaminases and synthetic functions after starting treatment.
At the time of enrollment to the study: 17 patients (29.3%) achieved biochemical remission with normalization of liver enzymes and 13 patients (22.4%) achieved both biochemical and immunological remission. Twenty-two patients (57.9%) out of 38 patients who achieved remission after initial presentation, developed relapses after normalization of their liver functions with median (IQR) of number of relapses 2 (2.5) range from 1 to 11 relapses. Most common cause of relapses was steroids withdrawal in 13 patients (59.1%) followed by non-compliance to ttt in 8 patients (36.4%). Sclerosing cholangitis was the cause of relapse in one patient (4.5%), Other 6 patients who had also sclerosing cholangitis did not achieved remission till time of enrollment to the study. None of the patients underwent liver transplantation.
Out of 58 patients of AIH, 28 patients (48.3%) had associated other autoimmune diseases. Seven patients had 2 associated ADs and one patient had 3 associated ADs thus raising the total number to 37 associated ADs in 28 patients. Autoimmune thyroiditis was the most common associated AD (17.2%). To screen for associated AIT; free T3, free T4, TSH, anti-TPO and anti-TG were done for all patients. Thyroid US and thyroid scan were done for patients with abnormal thyroid profile, autoantibodies or with borderline values. Out of 58 patients with AIH, 25 patients (43%) had thyroid abnormalities either in thyroid profile, autoantibodies, thyroid US or scan. Out of 25 patients with thyroid abnormalities, 10 patients (40%) were diagnosed as AIT.
Regarding thyroid status of all AIH children: 48 patients (82.8%) were euthyroid, 9 patients (15.5%) had subclinical hypothyroidism and one patient (1.7%) was hyperthyroid. Thyroid status of AIT patients showed that 6 patients (60%) were euthyroid, 3 patients (30%) had subclinical hypothyroidism and only one patient (10%) was hyperthyroid.
Finally, the screening for detection of AIT is recommended in all patients with AIH for early detection and for better prognosis.