Clinical and pathological characteristics and outcome of 46 children with autoimmune hepatitis.
Summary: This study reviewed and analyzed the clinical and pathological data of children with autoimmune hepatitis (AIH).
Conclusion: Autoimmune hepatitis-? is more common than autoimmune hepatitis-? in children, with diverse clinical characteristics. Most cases have progressive liver inflammation and fibrosis when diagnosed. Prednisolone monotherapy or combined with azathioprine could achieve both biochemical and pathological improvement, but relapse is inevitable during drug tapering, hence long-term treatment is essential.
Objective: To review and analyze the clinical and pathological data of children with autoimmune hepatitis (AIH).
Methods: Medical records of 46 patients hospitalized in Pediatric Liver Diseases Treatment and Research Center, Fifth Medical Center, General Hospital of People's Liberation Army(PLA) from April 2012 to April 2018 were extracted. Medical data included type of AIH, clinical manifestations, biochemical parameters, liver biopsy results, and outcomes of treatment were analyzed retrospectively. Among 46 children, 19 were males and 27 were females. The age of onset was 10.1(1.4-18.0) years old. Chi-Square test, Rank sum test or t test were used for inter-group comparison.
Results: There were 32 (70%)AIH-I cases and 14 (30%)AIH-Ⅱ cases (χ(2)=12.565, P=0.000). Among the 46 patients, there were 5 modes of onest: 17 cases (37%) had acute viral hepatitis-like presentation, 2 cases (4%) had fulminant hepatic failure, 9 cases (20%) had insidious onset, 5 cases (11%) showed cirrhosis and portal hypertension, and 13 cases (28%) were incidentally found to be due to elevated hepatic aminotransferases. Comorbidities including primary sclerotic cholangitis (n=3), primary biliary cholangitis (n=1), systemic lupus erythematosus (n=1) and inflammatory bowel disease (n=2), were all seen in AIH-Ⅰ cases. The elevated biochemical parameters of these patients were as follows: alanine aminotransferase (n=46), aspartate transminase (n=46), total bilirubin (n=35) γ-glutamyl transpeptadase (n=39), γ-globulin (n=32) and IgG (n=33). The γ-globulin and IgG levels were significantly higher in AIH-Ⅰ patients than those with AIH-Ⅱ((32±9)% vs. (23±8)%, t=3.217, P=0.002,(27±10) vs. (18±8)g/L, t=3.193, P=0.003, respectively). Thirty-nine patients received liver biopsy, among whom 22 (56%) with inflammation grade (G)≥3, 26(67%) with fibrosis stage (S) ≥3, and 7 with hepatic cirrhosis (S4) according to pathological analysis. Typical histopathological changes of AIH included: 36 cases of interfacial hepatitis (92%), 23 cases of lymphocyte/plasma cell infiltration (59%), 3 cases of rosette (8%). Forty patients received prednisolone monotherapy or combined with azathioprine after diagnosis. Complete remission was seen in 29 (72%) patients, partial remission in 10 (25%) patients and no response in 1 (3%) patient. Among complete remission patients, 15 (52%) had relapse in the process of prednisolone reduction. Repeated liver biopsy performed in 8 patients after treatment showed that hepatic inflammation and fibrosis were both improved in 6 patients, only inflammation was alleviated without fibrosis improvement in 1 patient, and neither inflammation nor fibrosis was improved in 1 case. The length of follow-up was 3.3 (0.3-10.5) years, and none of the 39 prednisolone-responded cases discontinued treatment successfully. Adverse effect of long-term prednisolone therapy included bilateral cataract (n=6), spinal fracture accompanied with delayed bone age development (n=1).
Conclusions: AIH-Ⅰ is more common than AIH-Ⅱ in children, with diverse clinical characteristics. Most cases have progressive liver inflammation and fibrosis when diagnosed. Prednisolone monotherapy or combined with azathioprine could achieve both biochemical and pathological improvement, but relapse is inevitable during drug tapering, hence long-term treatment is essential.