A rare etiology of persistent jaundice in type 1 autoimmune hepatitis

Budumuri Gautam V. Kumar, Purna Ch. Sethy, Rohit Gupta


A 24-year-old male without previously known comorbidities presented with progressive jaundice and hepatomegaly. Autoimmune serology and liver biopsy revealed features of autoimmune hepatitis. A treatment by oral prednisolone and azathioprine was initiated, after which he achieved remission. However, hyperbilirubinemia persisted, with a predominantly unconjugated fraction. Hemolytic causes of unconjugated hyperbilirubinemia were ruled out, and the diagnosis of Gilbert syndrome was established.


autoimmune hepatitis; hyperbilirubinemia; Gilbert syndrome

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Manns MP, Czaja AJ, Gorham JD, et al. Diagnosis and management of autoimmune hepatitis. Hepatology 2010; 51(6):2193-2213.

Czaja AJ, dos Santos RM, Porto A, Santrach PJ, Moore SB. Immune phenotype of chronic liver disease. Dig Dis Sci 1998; 43(9):2149-2155.

Bunchorntavakul C, Reddy KR. Diagnosis and management of overlap syndromes. Clin Liver Dis 2015; 19(1):81-97.

Waldenstrom J. Liver, blood proteins and nutritive protein. Dtsch Z Verdau Stoffwechselkr 1953; 9:113-119.

Manns MP, Lohse AW, Vergani D. Autoimmune hepatitis--Update 2015. J Hepatol 2015; 62(1):S100-S11.

Doumtsis P, Oikonomou T, Goulis I, Zachou K, Dalekos G, Cholongitas E. Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia. Ann Gastroenterol 2018; 31(1):123-126.

Czaja AJ. Diagnosis and Management of Autoimmune Hepatitis: Current Status and Future Directions. Gut Liver 2016; 10(2):177-203.

Vierling JM. Autoimmune Hepatitis and Overlap Syndromes: Diagnosis and Management. Clin Gastroenterol Hepatol 2015; 13(12):2088-2108.

Hennes EM, Zeniya M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48(1):169-176.

Lamers MMH, van Oijen MGH, Pronk M, Drenth JPH. Treatment options for autoimmune hepatitis: a systematic review of randomized controlled trials. J Hepatol 2010; 53(1):191-198.

Mack CL, Adams D, Assis DN, et al. Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the Study of Liver Diseases. Hepatology 2020; 72(2):671-722.

Wagner K-H, Shiels RG, Lang CA, Seyed Khoei N, Bulmer AC. Diagnostic criteria and contributors to Gilbert's syndrome. Crit Rev Clin Lab Sci 2018; 55(2):129-139.

Strassburg CP. Hyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin-Johnson, and Rotor syndrome). Best Pract Res Clin Gastroenterol 2010; 24(5):555-571.

Kamal S, Abdelhakam S, Ghoraba D, et al. The frequency, clinical course, and health related quality of life in adults with Gilbert's syndrome: a longitudinal study. BMC Gastroenterol 2019; 19(1):22.

DOI: http://dx.doi.org/10.22551/2021.32.0803.10183

Copyright (c) 2021 Budumuri Gautam V. Kumar, Purna Ch. Sethy, Rohit Gupta

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ISSN: 2360-6975