Primary sclerosing cholangitis in children with inflammatory bowel disease

Abstract

Introduction. Currently, in pediatric practice, there are no clear data on the features of the onset of primary sclerosing cholangitis (PSC) in inflammatory bowel diseases (IBD), the frequency of detection of markers of cytolysis and cholestasis, the nature of the lesion of the hepatobiliary system and changes in the endoscopic picture. Purpose: assessment of clinical, laboratory, and instrumental features of PSC course in IBD children (PSC+IBD). Materials and methods. For the period 2015-2021, a comprehensive examination of 54 children with PSC+IBD (main group) and 79 patients with isolated IBD (comparison group): ulcerative colitis (UC) or Crohn’s disease (CD) was carried out. Results. In 54 children of the main group, the combination of PSC+UC was detected in 48 (88.9%) patients with a predominance of boys. At the onset of the disease, the most frequent manifestations in children with PSC+IBD were diarrhea, abdominal pain syndrome, cytolysis and cholestasis syndromes, and hemocolitis. In children with PSC+IBD, compared with patients with IBD, there was a statistically significant increase in serum concentrations of ALT, AST and total protein, indicating an increase in hepatitis activity; GGT, alkaline phosphatase and direct bilirubin - about an increase in the severity of cholestasis syndrome; IgG - as a marker of the severity of mesenchymal-inflammatory syndrome in the framework of liver damage. According to the endoscopic data, with PSC+UC, pancolitis with more pronounced involvement of the right colon or isolated proctitis prevails, and with PSC+CD, ileocolitis is more common. Indices PUCAI (p < 0.001) and PCDAI (p = 0.027) were statistically significantly lower in the PSC+IBD group than in the isolated IBD. According to the data of transient liver elastometry, no statistically significant difference was obtained between the medians of the parameters of the biochemical blood test at different stages of fibrosis. According to the results of magnetic resonance imaging of the abdominal organs and MR-cholangiopancreatography, the most characteristic changes in PSC+IBD are heterogeneity of the liver parenchyma, unevenness of the intra- and extrahepatic bile ducts, the presence of areas of expansion and narrowing of their diameter. Conclusions. The phenotype of PSC+IBD in children is manifested mainly by intestinal symptoms, more often UC with involvement of the right parts of the intestine in boys and a lower activity index than the isolated course of IBD, in combination with laboratory syndromes and cholestasis.