Can liver biopsy be spared for the diagnosis of autoimmune hepatitis in selected children? A multicenter retrospective study

Autoimmune hepatitis (AIH) is diagnosed based on clinical, biochemical, immunological, and histological parameters, and on the exclusion of other liver diseases. Multiple scoring systems are available for AIH diagnosis, all of which require liver biopsy (LB). With the aim of reducing invasive procedures to minimize patient’s risks, this study evaluated whether LB may be spared for AIH diagnosis in some children, similar to primary biliary cholangitis. Children with histologically confirmed autoimmune liver disease (AILD) were evaluated from 5 Pediatric Units. We retrospectively collected clinical, laboratory, imaging and histological data to assess AIH diagnostic scores (International Autoimmune Hepatitis Group [IAIHG] criteria, juvenile AIH score [JAIH], and simplified criteria [s-IAIHG]) in each patient pre- and post-LB. The diagnosis of autoimmune sclerosing cholangitis (ASC) was based on magnetic resonance cholangiopancreatography and liver histology. Ninety-one patients (55 females) were evaluated (36 with AIH type I, 24 with AIH type II, 8 with seronegative AIH, and 23 with ASC). The mean age at diagnosis and duration of follow-up were 8.9±4.8 and 9.6±7.8 years, respectively. Based on IAIHG, JAIH and s-IAIHG scores, pre-LB scores were “definite” for 15.3%, 49.5%, and 0% of patients, respectively. Post-LB, the diagnosis was confirmed in all these patients. We found no associations between liver histological findings or diagnostic scores and relapses or treatment withdrawal. In patients with a “definite” AIH score pre-LB, LB is not necessary for diagnosis. Histological findings and scoring systems do not predict relapses or treatment withdrawal.