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


CONCLUSIONS
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.
Radhakrishnan
Kadakkal R
KR
Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States.
Alkhouri
Naim
N
Worley
Sarah
S
Arrigain
Susana
S
Hupertz
Vera
V
Kay
Marsha
M
Yerian
Lisa
L
Wyllie
Robert
R
Feldstein
Ariel E
AE
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D014481:United States / Q000453:epidemiology