Published online Dec 27, 2021. doi: 10.4254/wjh.v13.i12.2113
Peer-review started: May 27, 2021
First decision: July 6, 2021
Revised: July 7, 2021
Accepted: November 25, 2021
Article in press: November 25, 2021
Published online: December 27, 2021
Previous reports showed some beneficial effect of oral vancomycin treatment (OVT) in children with primary sclerosing cholangitis; conversely, the experience in patients with other autoimmune liver diseases (AILD), including autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC), is scant.
To assess the response to immunosuppressive treatment (IS) and to OVT in children diagnosed with AILD.
Retrospective study of children diagnosed with AIH (normal biliary tree at cholangiography) and ASC (abnormal biliary tree at cholangiography) in the last 10 years. All underwent standard immunosuppressive therapy (IS), but non-responders received also OVT. Biochemical remission [normal aspartate aminotransferase (AST)] and immunological remission (normal IgG and negative autoantibodies) rates and Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) index were assessed and compared during the follow up.
75 children were included [69% female, median age 10.5 years (5.6-13.4 years), AIH = 54, ASC= 21]. Sixty-three patients (84%, AIH = 52, ASC = 11) were treated with standard IS and 61 achieved biochemical remission, whereas 12 not responding to IS [16%, F = 75%, median age 13.5 years, (12.2-15.7), 10 with ASC] required OVT and 8 achieved biochemical remission. Overall OVT increased the biochemical remission rate of the whole group of AILD patients from 81% (61/75) to 92% (69/75). Median values of AST, alanine aminotransferase (ALT) and gamma-glutamyl transferase (GGT) decreased significantly after OVT start (P < 0.05). Complete normalization of livers enzymes (AST, ALT and GGT) was observed in 6/12 patients (50%). Decrease in SCOPE index score was reported in 5/12 patients (42%). At last follow up (median of 4.4 years, range 0.6-13.8 years) all 75 patients are alive, 6 (8%, 1 with ASC) successfully discontinued medications, 1 (with ASC) required liver transplantation.
Children with AIH and ASC respond well to IS treatment. OVT may represent a valuable treatment option to achieve biochemical remission in patients not responding to standard IS. These promising preliminary results suggest that a prospective study is indicated to define the efficacy of OVT in AILD.
Core Tip: Experience with oral vancomycin in children with autoimmune liver disease (AILD) is limited. We enrolled 75 children [median age 10.5 years (5.6-13.4)], 54 with autoimmune hepatitis and 21 with autoimmune sclerosing cholangitis; 63/75 achieved remission by standard immunosuppressive therapy (IS), whereas 12/75 (16%) required oral vancomycin treatment (OVT). In 6/12 patients (50%) the response was complete, whereas it was partial in 2/12 (17%), and absent in 4/12 (33%). Overall OVT increased the remission rate of the whole group of AILD patients from 81% to 92%. OVT may represent a valuable treatment option in children with AILD who do not respond to standard IS.