Angelo Di Giorgio1, A D'Adda2, A Marseglia2, A Sonzogni3, L Licini3, E Nicastro2, L D'Antiga2. 1. Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Piazza Oms 1, 24127, Bergamo, Italy. adigiorgio@asst-pg23.it. 2. Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Piazza Oms 1, 24127, Bergamo, Italy. 3. Liver Pathology, Hospital Papa Giovanni XXIII, Bergamo, Italy.
Abstract
OBJECTIVES AND STUDY: Various degrees of biliary changes are considered to be part of the histological picture of children with pediatrics autoimmune liver disease (AILD), but the literature is scarce and confusing. We aimed to describe the characteristics of children with AILD (autoimmune hepatitis, AIH, and autoimmune sclerosing cholangitis, ASC) focusing on the prevalence and type of biliary abnormalities on initial biopsy to see whether ASC was predictable on histological ground. METHODS: The files of children diagnosed with AILD were reviewed. The Ishak score was used to grade inflammation and fibrosis on biopsy; a biliary score was built to grade bile duct injury. Demographic, laboratory and histological features at diagnosis were reported and compared between the two groups (AIH vs ASC). RESULTS: Forty-one patients were diagnosed with AIH (n = 24), ASC (n = 13) and PSC (n = 4) between 2009 and 2018. Twenty-nine patients [F = 76%, AIH = 20, ASC = 9, median age at diagnosis 11.7 (range 2.2-17.8)] were included in the study; 12 (4 with PSC) were excluded. Prevalence of inflammatory bowel disease was higher in ASC group (56% vs 10% in AIH, p < 0.05). On histology 17% had cirrhosis. The grade of biliopathy with AILD was moderate in 72% and severe in 31%, and overall more prominent in ASC (p = 0.031). The inflammation of the bile ducts was classified as "multifocal" or "diffuse" mainly in ASC patients (89% vs 45% in AIH, p = 0.043). Periductular fibrosis was reported in 52% of AILD patients, with a higher mean score in ASC group (p < 0.05). However, ductular reaction, biliary metaplasia and granulomatous cholangitis were equally reported in AIH and ASC, providing no clear-cut for the distinction of the two entities in the global histological evaluation. CONCLUSIONS: Majority of patients with pediatrics AILD have "moderate" or "severe" features of biliopathy; AIH and ASC are not easily distinguishable on histological ground at diagnosis, and therefore, the cholangiogram remains the only effective tool to differentiate patients with AIH from those with ASC. Further prospective studies are needed to better define histological biliary features in AILD, assess if the biliopathy responds to immunosuppressive treatment and evaluate its impact on long-term outcome.
OBJECTIVES AND STUDY: Various degrees of biliary changes are considered to be part of the histological picture of children with pediatrics autoimmune liver disease (AILD), but the literature is scarce and confusing. We aimed to describe the characteristics of children with AILD (autoimmune hepatitis, AIH, and autoimmune sclerosing cholangitis, ASC) focusing on the prevalence and type of biliary abnormalities on initial biopsy to see whether ASC was predictable on histological ground. METHODS: The files of children diagnosed with AILD were reviewed. The Ishak score was used to grade inflammation and fibrosis on biopsy; a biliary score was built to grade bile duct injury. Demographic, laboratory and histological features at diagnosis were reported and compared between the two groups (AIH vs ASC). RESULTS: Forty-one patients were diagnosed with AIH (n = 24), ASC (n = 13) and PSC (n = 4) between 2009 and 2018. Twenty-nine patients [F = 76%, AIH = 20, ASC = 9, median age at diagnosis 11.7 (range 2.2-17.8)] were included in the study; 12 (4 with PSC) were excluded. Prevalence of inflammatory bowel disease was higher in ASC group (56% vs 10% in AIH, p < 0.05). On histology 17% had cirrhosis. The grade of biliopathy with AILD was moderate in 72% and severe in 31%, and overall more prominent in ASC (p = 0.031). The inflammation of the bile ducts was classified as "multifocal" or "diffuse" mainly in ASC patients (89% vs 45% in AIH, p = 0.043). Periductular fibrosis was reported in 52% of AILD patients, with a higher mean score in ASC group (p < 0.05). However, ductular reaction, biliary metaplasia and granulomatous cholangitis were equally reported in AIH and ASC, providing no clear-cut for the distinction of the two entities in the global histological evaluation. CONCLUSIONS: Majority of patients with pediatrics AILD have "moderate" or "severe" features of biliopathy; AIH and ASC are not easily distinguishable on histological ground at diagnosis, and therefore, the cholangiogram remains the only effective tool to differentiate patients with AIH from those with ASC. Further prospective studies are needed to better define histological biliary features in AILD, assess if the biliopathy responds to immunosuppressive treatment and evaluate its impact on long-term outcome.
Authors: Diego Vergani; Fernando Alvarez; Francesco B Bianchi; Eduardo L R Cançado; Ian R Mackay; Michael P Manns; Mikio Nishioka; Edward Penner Journal: J Hepatol Date: 2004-10 Impact factor: 25.083
Authors: Michael P Manns; Albert J Czaja; James D Gorham; Edward L Krawitt; Giorgina Mieli-Vergani; Diego Vergani; John M Vierling Journal: Hepatology Date: 2010-06 Impact factor: 17.425
Authors: Roger Chapman; Johan Fevery; Anthony Kalloo; David M Nagorney; Kirsten Muri Boberg; Benjamin Shneider; Gregory J Gores Journal: Hepatology Date: 2010-02 Impact factor: 17.425
Authors: G V Gregorio; B Portmann; J Karani; P Harrison; P T Donaldson; D Vergani; G Mieli-Vergani Journal: Hepatology Date: 2001-03 Impact factor: 17.425
Authors: Kirsten Muri Boberg; Roger W Chapman; Gideon M Hirschfield; Ansgar W Lohse; Michael P Manns; Erik Schrumpf Journal: J Hepatol Date: 2010-09-18 Impact factor: 25.083
Authors: Elke M Hennes; Mikio Zeniya; Albert J Czaja; Albert Parés; George N Dalekos; Edward L Krawitt; Paulo L Bittencourt; Gilda Porta; Kirsten M Boberg; Harald Hofer; Francesco B Bianchi; Minoru Shibata; Christoph Schramm; Barbara Eisenmann de Torres; Peter R Galle; Ian McFarlane; Hans-Peter Dienes; Ansgar W Lohse Journal: Hepatology Date: 2008-07 Impact factor: 17.425
Authors: Kadakkal R Radhakrishnan; Naim Alkhouri; Sarah Worley; Susana Arrigain; Vera Hupertz; Marsha Kay; Lisa Yerian; Robert Wyllie; Ariel E Feldstein Journal: Dig Liver Dis Date: 2010-02-16 Impact factor: 4.088
Authors: Angelo Di Giorgio; Anna Tulone; Emanuele Nicastro; Lorenzo Norsa; Aurelio Sonzogni; Lorenzo D'Antiga Journal: World J Hepatol Date: 2021-12-27