Incidence and clinical features of autoimmune hepatitis in the province of santa fe (Argentina)

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To investigate the incidence and clinical features of autoimmune hepatitis (AIH) in children from the province of Santa Fe, Argentina, over ten years.


From the records of all of the pediatric hepatologists in the province of Santa Fe, Argentina, we reviewed the clinical charts of patients < 18 years who were diagnosed with AIH (simplified score >6 points) and followed between January 2003 and December 2013. Population data were extracted from the 2010 national census. Values were expressed as percentages and median +/- interquartile range. Mann-Whitney test was used for comparison between groups.


67 patients fulfilled inclusion criteria, from which 11 (16%) were later reclassified as “autoimmune sclerosing cholangitis” (ASC) according to biochemical, histological and radiological findings. A final sample of 56 patients (39 F) with AIH was analyzed, giving an annual incidence of 0.56/100,000. Median age at presentation was 8 (5.7 -11) years, and the median follow-up was 4 (2–7) years. Type 1 AIH was diagnosed in 89%. An acute presentation was observed in 53%, while 13 (23%) showed cirrhosis on initial biopsy. Prednisone (87%), and azathioprine (60%) were the most common drugs prescribed. At the end of follow-up, 53/56 (95%) were alive, including four patients (7%) who underwent liver transplantation.


AIH has an estimated incidence of 0.56/100000/year in children from the province of Santa Fe (Argentina). Overall survival was 95%. A subgroup of patients diagnosed as AIH develops predominant biliary disease and should be better classified as ASC.

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