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

Radhakrishnan, Kadakkal R.; Alkhouri, Naim; Worley, Sarah; Arrigain, Susana; Hupertz, Vera; Kay, Marsha; Yerian, Lisa; Wyllie, Robert; Feldstein, Ariel E.

doi:10.1016/j.dld.2010.01.002

« Previous Next »Digestive and Liver Disease
Volume 42, Issue 10 , Pages 724-728, October 2010

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

Kadakkal R. Radhakrishnan
- Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States
Naim Alkhouri
- Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States
- Corresponding author. Tel.: +1 216 444 9000; fax: +1 216 444 2974.
Sarah Worley
- Department of Qualitative Health Sciences, Cleveland Clinic, Cleveland, OH, United States
Susana Arrigain
- Department of Qualitative Health Sciences, Cleveland Clinic, Cleveland, OH, United States
Vera Hupertz
- Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States
Marsha Kay
- Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States
Lisa Yerian
- Department of Anatomical Pathology, Cleveland Clinic, Cleveland, OH, United States
Robert Wyllie
- Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States
Ariel E. Feldstein
- Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States
- Department of Cell Biology, Cleveland Clinic, Cleveland, OH, United States

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

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.

Keywords: Autoimmune hepatitis, Primary sclerosing cholangitis, Cirrhosis, Outcome