Sclerosing cholangitis in children with inflammatory bowel disease

Česká verzia

Authors: Vlčková Eva; Mitrová Katarína
Authors place of work: Pediatrická klinika 2. lékařské fakulty Univerzity Karlovy a Fakultní nemocnice v Motole
Published in the journal: Čes-slov Pediat 2023; 78 (3): 182-186.
Category: Review
doi: https://doi.org/10.55095/CSPediatrie2023/025

Summary

Primary sclerosing cholangitis is a chronic progressive disease of the biliary tract. There are many differences between adult primary sclerosing cholangitis and juvenile type. Major part of pediatric PSC patients will develop inflammatory bowel disease (IBD). The phenotype of IBD in patients with PSC-IBD differs from those without associated PSC and is characterized by a milder course and typical findings of pancolitis, rectal sparing and backwash ileitis. Endoscopic activity of the disease is more often present despite the absence of symptoms suggesting subclinical inflammation. The course of PSC associated with Crohn’s disease is usually less severe than that with ulcerative colitis (UC). Compared to patients with an isolated form of UC, patients with PSC-UC are more often treated with amino-salicylates monotherapy. Likewise, these patients have been reported to have a lower risk of undergoing colectomy or requiring steroids, thiopurines, or biologic therapy than patients without associated PSC. Recent data suggest that none of the current treatment modalities can stop the progression of PSC, and significant number of patients will end up with liver failure requiring a liver transplant.

Keywords:

therapy – children – primary sclerosing cholangitis (PSC) – inflammatory bowel disease (IBD)

Zdroje

1. Ricciuto A, Kamath BM, Griffiths AM. The IBD and PSC phenotypes of PSC-IBD. Curr Gastroenterol Rep 2018; 20(4): 16.
2. Kerkar N, Chan A. Autoimmune hepatitis, sclerosing cholangitis, and autoimmune sclerosing cholangitis or overlap syndrome. Clin Liver Dis 2018; 22(4): 689–702.
3. Mieli-Vergani G, Vergani D, Baumann U, et al. Diagnosis and management of pediatric autoimmune liver disease: ESPGHAN Hepatology Committee Position Statement 2018; 66(2): 345–360.
4. Deneau MR, El-Matary W, Valentino PL, et al. The natural history of primary sclerosing cholangitis in 781 children. Hepatology 2017; 66(2): 518–527.
5. Lunder AK, Hov JR, Borthne A, et al. Prevalence of sclerosing cholangitis detected by magnetic resonance cholangiography in patients with long-term inflammatory bowel disease. Gastroenterology 2016; 151(4): 660–669.
6. Laborda TJ, Jensen MK, Kavan M, et al. Treatment of primary sclerosing cholangitis in children. World J Hepatol 2019; 11(1): 19–36.
7. Ji S, Juran BD, Mucha S, et al. Genome-wide association study of primary sclerosing cholangitis identifies new risk loci and quantifies the genetic relationship with inflammatory bowel disease. Nat Genet 2017; 49(2): 269–273.
8. Loftus EV. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005; 54(1): 91–96.
9. Bajer L, Kamenář D, Sticová E, et al. Idiopatický střevní zánět u pacientů s primární sklerozující cholangitidou – samostatný fenotyp IBD. Gastroent Hepatol 2014; 68(1): 24–35.
10. Ricciuto A, Hansen BE, Ngo B, et al. Primary sclerosing cholangitis in children with inflammatory bowel diseases is associated with milder clinical activity but more frequent subclinical inflammation and growth impairment. Clin Gastroenterol Hepatol 2020; 18(7): 1509–1517.
11. Sokol H, Cosnes J, Chazouilleres O, Beaugerie L, et al. Disease activity and cancer risk in inflammatory bowel disease associated with primary sclerosing cholangitis. World J Gastroenterol 2008; 14(22): 3497–3503.
12. Bramuzzo M, Martelossi S, Torre G, et al. Clinical features and risk factors of autoimmune liver involvement in pediatric inflammatory bowel disease. J Pediatr Gastroenterol Nutr 2016; 63(2): 259–264.
13. Zheng H, Jiang X. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease. Eur J Gastroenterol Hepatol 2016; 28(4): 383–390.
14. Lindström L, Jørgensen KK, Boberg KM, et al. Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation: a Nordic Multicentre Study. Scandinavian J Gastroenterol 2018; 53(3): 297–304.
15. Nevoral J, et al. Praktická pediatrická gastroenterologie, hepatologie a výživa. Praha: Mladá fronta 2013.
16. Bajer L, Wohl P, Drastich P. PSC-IBD: specifický fenotyp idiopatického střevního zánětu asociovaný s primární sklerózující cholangitidou. Vnitř Lék 2018; 64(6): 659–664.
17. Turner D, Ruemmele FM, Orlanski-Meyer E, et al. Management of paediatric ulcerative colitis. Part 1: Ambulatory care-an evidence-based guideline from European Crohn’s and Colitis Organization and European Society of Paediatric Gastroenterology, Hepatology and Nutrition. JPGN 2018; 67(2):257–291.
18. Laborda TJ, Ricciuto A, Aumar M, et al. Vedolizumab therapy in children with primary sclerosing cholangitis: Data from the Pediatric Primary Sclerosing Cholangitis Consortium. JPGN 2020; 71(4): 459–464.
19. Deneau MR, Perito E, Ricciuto A, et al. Ursodeoxycholic acid therapy in pediatric primary sclerosing cholangitis: predictors of gamma glutamyltransferase normalization and favorable clinical course. J Pediatr 2019; 209:92–96.
20. Deneau MR, Mack C, Mogul D, et al. Oral vancomycin, ursodeoxycholic acid, or no therapy for pediatric primary sclerosing cholangitis: a matched analysis. Hepatology 2021; 73(3): 1061–1073.
21. Di Giorgio A, Tulone A, Nicastro E, et al. Use of oral vancomycin in children with autoimmune liver disease: A single centre experience. World J Hepatol 2021; 13(12): 2113–2127.
22. Ponsioen CY, Arnelo U, Berquist A, et al. No superiority of stents vs balloon dilatation for dominant strictures in patients with primary sclerosing cholangitis. Gastroenterology 2018; 155(3): 752–759.