Real-world Management of Juvenile Autoimmune Liver Disease

Overview

Journal United European Gastroenterol J

Publisher Wiley

Specialty Gastroenterology

Date 2018 Sep 20

PMID 30228891

Citations 3

Authors

Y S de Boer

R Liberal

D Vergani

G Mieli-Vergani

Affiliations

Soon will be listed here.

Abstract

Background And Aims: Juvenile autoimmune liver disease (JAILD) includes paediatric forms of autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC). Since evidence is scarce, there are currently no evidence-based management guidelines for juvenile AIH. This survey was carried out amongst the paediatric members of the International AIH Group (IAIHG) to describe their practices in the management of JAILD.

Methods: An online survey questionnaire was distributed to members of the IAIHG with active practice (https://www.surveymonkey.de/r/Juvenile_AILD). The questionnaire consisted of four clinical scenarios on different presentations of AIH.

Results: Fifty-eight surveys were sent to the IAIHG members, out of which 43 (74%, 22 countries, four continents) were returned. None reported budesonide as a first-line induction agent for the acute presentation of AIH. Sixteen (37%) routinely perform liver biopsy at three years of biochemical remission. Thirty-five respondents (81%) perform magnetic resonance cholangiography (MRC) at presentation. Ciclosporin is the most widely used second-line agent (number of patients treated = ∼360, 21 centres). Mycophenolate mofetil ( = ∼225, 31 centres), tacrolimus ( = ∼130, 21 centres) and sirolimus ( = ∼5, 3 centres) are less often reported. Rescue therapy with infliximab and rituximab has been tried in eight centres ( = ∼19) and nine centres ( = ∼16), respectively.

Conclusions: Prednisolone remains the preferred first-line induction agent in JAILD. MRC at presentation is performed by the large majority of participants. Participants reported a wide variation in performing liver biopsy for therapy evaluation during follow-up. Within the paediatric members of the IAIHG there is considerable experience with second-line therapeutic agents.

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References

Garner S, Eady A, Bennett C, Newton J, Thomas K, Popescu C . Minocycline for acne vulgaris: efficacy and safety. Cochrane Database Syst Rev. 2012; (8):CD002086. PMC: 7017847. DOI: 10.1002/14651858.CD002086.pub2. View

Eichenfield A . Minocycline and autoimmunity. Curr Opin Pediatr. 1999; 11(5):447-56. DOI: 10.1097/00008480-199910000-00014. View

Alvarez F, Berg P, Bianchi F, Bianchi L, Burroughs A, Cancado E . International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999; 31(5):929-38. DOI: 10.1016/s0168-8278(99)80297-9. View

Manns M, Czaja A, Gorham J, Krawitt E, Mieli-Vergani G, Vergani D . Diagnosis and management of autoimmune hepatitis. Hepatology. 2010; 51(6):2193-213. DOI: 10.1002/hep.23584. View

Lan J, Lahoti A, Lew D . A severe case of minocycline-induced DRESS resulting in liver transplantation and autoimmune sequelae. Ann Allergy Asthma Immunol. 2016; 116(4):367-8. DOI: 10.1016/j.anai.2015.12.010. View

de Boer Y, Kosinski A, Urban T, Zhao Z, Long N, Chalasani N . Features of Autoimmune Hepatitis in Patients With Drug-induced Liver Injury. Clin Gastroenterol Hepatol. 2016; 15(1):103-112.e2. PMC: 5370577. DOI: 10.1016/j.cgh.2016.05.043. View

Ang G, Simpson S, Reddy A . Mycophenolate mofetil embryopathy may be dose and timing dependent. Am J Med Genet A. 2008; 146A(15):1963-6. DOI: 10.1002/ajmg.a.32420. View

Krawitt E . Autoimmune hepatitis. N Engl J Med. 2006; 354(1):54-66. DOI: 10.1056/NEJMra050408. View

Mieli-Vergani G, Heller S, Jara P, Vergani D, Chang M, Fujisawa T . Autoimmune hepatitis. J Pediatr Gastroenterol Nutr. 2009; 49(2):158-64. DOI: 10.1097/MPG.0b013e3181a1c265. View

10.

Gregorio G, Portmann B, Reid F, Donaldson P, Doherty D, McCartney M . Autoimmune hepatitis in childhood: a 20-year experience. Hepatology. 1997; 25(3):541-7. DOI: 10.1002/hep.510250308. View

11.

. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol. 2015; 63(4):971-1004. DOI: 10.1016/j.jhep.2015.06.030. View

12.

Liberal R, de Boer Y, Andrade R, Bouma G, Dalekos G, Floreani A . Expert clinical management of autoimmune hepatitis in the real world. Aliment Pharmacol Ther. 2016; 45(5):723-732. DOI: 10.1111/apt.13907. View

13.

Liberal R, Grant C, Ma Y, Csizmadia E, Jiang Z, Heneghan M . CD39 mediated regulation of Th17-cell effector function is impaired in juvenile autoimmune liver disease. J Autoimmun. 2016; 72:102-12. PMC: 6348153. DOI: 10.1016/j.jaut.2016.05.005. View

14.

Zizzo A, Valentino P, Shah P, Kamath B . Second-line Agents in Pediatric Patients With Autoimmune Hepatitis: A Systematic Review and Meta-analysis. J Pediatr Gastroenterol Nutr. 2017; 65(1):6-15. DOI: 10.1097/MPG.0000000000001530. View

15.

Carbone M, Neuberger J . Autoimmune liver disease, autoimmunity and liver transplantation. J Hepatol. 2013; 60(1):210-23. DOI: 10.1016/j.jhep.2013.09.020. View

16.

Manns M, Woynarowski M, Kreisel W, Lurie Y, Rust C, Zuckerman E . Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis. Gastroenterology. 2010; 139(4):1198-206. DOI: 10.1053/j.gastro.2010.06.046. View

17.

Gregorio G, Portmann B, Karani J, Harrison P, Donaldson P, Vergani D . Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001; 33(3):544-53. DOI: 10.1053/jhep.2001.22131. View

18.

Woynarowski M, Nemeth A, Baruch Y, Koletzko S, Melter M, Rodeck B . Budesonide versus prednisone with azathioprine for the treatment of autoimmune hepatitis in children and adolescents. J Pediatr. 2013; 163(5):1347-53.e1. DOI: 10.1016/j.jpeds.2013.05.042. View

19.

Perez-Aytes A, Marin-Reina P, Boso V, Ledo A, Carey J, Vento M . Mycophenolate mofetil embryopathy: A newly recognized teratogenic syndrome. Eur J Med Genet. 2016; 60(1):16-21. DOI: 10.1016/j.ejmg.2016.09.014. View

20.

Mieli-Vergani G, Vergani D . Autoimmune hepatitis. Nat Rev Gastroenterol Hepatol. 2011; 8(6):320-9. DOI: 10.1038/nrgastro.2011.69. View