Association of autoimmune hepatobiliary pathology with inflammatory bowel diseases in children

Abstract

Introduction. Hepatobiliary pathology (HBP) occurs in approximately 30% of patients with inflammatory bowel disease (IBD). However, the features of its course in the pediatric cohort of patients remain insufficiently studied. Purpose: to study the features of the course of autoimmune forms of HBD in children with IBD. Materials and methods. A comprehensive clinical, laboratory and instrumental examination was carried out in 84 children with autoimmune forms of HBP in combination with IBD (HBP+IBD), which made up the main group, and 79 patients with isolated forms of IBD included in the comparison group. Results. The prevalence of autoimmune HBP in IBD children was 10.2%. Primary sclerosing cholangitis (PSC) was diagnosed in 64.3% of cases, which was mainly associated with ulcerative colitis. The incidence of autoimmune hepatitis (AIH) was 8.3%. In the structure of the overlap syndrome, the most frequent combination was AIH+PSC (15.5%). The debut of the disease was manifested by diarrhea, abdominal pain syndrome, cytolysis and cholestasis syndromes, haemicolitis. With HBP+IBD, there was an increase in serum concentrations of alanine (ALT) and aspartate aminotransferases (AST), total protein, γ-glutamyl transferase (GGT), alkaline phosphatase, direct bilirubin and IgG. Approximately with the same frequency in PSC, antibodies to saccharomycetes (ASCA) - 80% and antibodies to the cytoplasm of neutrophils (ANCA) - 75% were detected. In AIH, antinuclear antibodies (ANA) and antibodies to liver and kidney microsomes (anti-LKM1) were detected in 100%. HBP-IBD equally (28.6%) revealed moderate fibrosis and cirrhosis, no fibrosis in 20.6%, moderate fibrosis in 15.9% of cases, mild fibrosis in 6,3%. Cirrhosis of the liver in 55.6% of cases was the outcome of the course of PSC, in 16.7% - AIH, in 27.8% was associated with the course of the overlap syndrome. Conclusion. Various forms of autoimmune HBP occur in 10.2% of cases, are more often associated with UC, are represented by PSC and AIH, occur in males, at the onset signs are clinically presented by diarrhea, abdominal pain syndrome, cytolysis and cholestasis syndromes, and haemicolitis.