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Autoimmune hepatitis in children—Impact of cirrhosis at presentation on natural history and long-term outcome

K.R. Radhakrishnana, N. AlkhouriaCorresponding Author Informationemail address, S. Worleyb, S. Arrigainb, V. Hupertza, M. Kaya, L. Yerianc, R. Wylliea, A.E. Feldsteinad

Received 27 July 2009; accepted 11 January 2010. published online 17 February 2010.
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Abstract 

Little is known regarding the natural history of autoimmune hepatitis in children. The aims of this longitudinal cohort study were to determine the long-term prognosis of children with autoimmune hepatitis and to determine the effect of cirrhosis at presentation on survival.

Methods

Thirty-three children with autoimmune hepatitis who were seen at our institution over a 25-year period were studied retrospectively.

Results

The median age of diagnosis was 12.9 years (2.7–18.1) with a female predominance of 3:1. Liver biopsies showed cirrhosis in 18 (55%) patients at time of diagnosis. Patients with cirrhosis at baseline had a similar 10-year survival 85% (70–100%) to those without cirrhosis 75% (49–100%) (p=0.97). The overall survival was significantly lower than the expected in the age- and gender-matched U.S. population (log-rank test; p<0.001). In Cox regression models, weight loss (p=0.037), baseline elevated bilirubin (p=0.028), prolonged International Normalized Ratio (INR) (p=0.013), and positive LKM-1 antibodies (p=0.007) were associated with shorter survival.

Conclusion

AIH in children is associated with a significant shorter survival rate than the expected in the general population. Cirrhosis on initial liver biopsy does not seem to impact long-term survival in children with AIH.

a Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States

b Department of Qualitative Health Sciences, Cleveland Clinic, Cleveland, OH, United States

c Department of Anatomical Pathology, Cleveland Clinic, Cleveland, OH, United States

d Department of Cell Biology, Cleveland Clinic, Cleveland, OH, United States

Corresponding Author InformationCorresponding author. Tel.: +1 216 444 9000; fax: +1 216 444 2974.

PII: S1590-8658(10)00006-X

doi:10.1016/j.dld.2010.01.002