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Case 312


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17 year-old male patient comes from Carmo da Mata (Minas Gerais) to the EC unit after hospitalisation due to hematemesis, melena, altered hepatic function and low level of platelets. No other comorbidities. Denies alcohol intake, smoking or use of recreational drugs. Denies any contact with fresh water. Physical examination reveals hepatomegaly and pain during right hypochondrium palpation. Requestes Upper Gastrointestinal Endoscopy (Image 1) and laboratorial review (Image 2) to diagnostic elucidation.

Considering the clinical data, the results and that viral serology were negative to Hepatitis B and C, which one is the most likely diagnosis?

a) Wilson's disease


b) Primary sclerosing cholangitis


c) Type 1 autoimmune hepatitis


d) Type 2 autoimmune hepatitis


Image analysis

Image 1 - Analysis: Upper Gastrointestinal Endoscopy, picture of the oesophagus showing small and medium-size esophageal varices (red arrows) due to portal hypertension.


- Autoimmune Hepatitis (AIH) is a chronic affection leading to progressive destruction of liver parenchyma. It must be suspected in young patients with chronic hepatopathy and negative viral serology;

- Type 1 AIH is positive to ASMA and AAN, while type 2 is positive to Anti-LKM1;

- Diagnostic criteria include: positive autoantibodies, elevated transaminases, hypergammaglobulinemia and high levels of IgG;

- 1st line treatment includes prednisone with or without azathioprine, showing good prognosis;

- Treatment is suspended after 18 months of clinical and histological remission, although most cases presents recidives.


- Couto C, Bittencourt P. Controvérsias no diagnóstico e tratamento da hepatite auto-imune. In: Savassi-Rocha P, Coelho L, Sanchez M, Rausch M, ed. by. Tópicos em Gastroenterologia 14 - Controvérsias. 1st ed. Rio de Janeiro: Guanabara Koogan; 2015.

- Santos BC, Campos MLP, Couto CA. Hepatopatias Crônicas Não-Virais. In: Santos BC, Faria IM, Couto CA. Manual Diagnóstico em Gastroenterologia para o Estudante de Medicina. 1st ed. Belo Horizonte: GASTROliga; 2018.

- Blumberg, S., Burakoff, Robertgreenberger, Norton, J. Current diagnosis & treatment. Gastroenterology, hepatology, & endoscopy. 3rd ed. New York: McGraw-Hill, 2016.

- Santos R, Alissa F, Reyes J, Teot L, Ameen N. Fulminant hepatic failure: Wilson's disease or autoimmune hepatitis? Implications for transplantation. Pediatric Transplantation. 2005;9(1):112-116.

- Santos B, Guedes L, Faria L, Couto C. Wilson’s disease presentation resembling autoimmune hepatitis: case report. In press.


Bruno Campos Santos, 4th year medical student at Universidade Federal de Minas Gerais.

Email: bruno_campos[at]

Fernando Henrique Melo Amorim Pinto, 4th year medical student at Universidade Federal de Minas Gerais.

Email: 12fernandoamorim[at]

Mateus Jorge Nardelli, 3rd year medical student at Universidade Federal de Minas Gerais.

Email: mateusnardelli[at]


Claudia Alves Couto, MD, Ph.D., Associated Professor and Doctor of the Department of Medical Clinic at the Medical School of Universidade Federal de Minas Gerais.

Email: clalcouto[at]


Lucas Bruno Rezende, Luana Almeida, Daniela Tereza Gonçalves Manso, Izabella Costa, Luanna Monteiro, MD, Viviane Santuari Parisotto Marino, MD, Ph.D.

Translated by

Bruno Campos Santos, 4th year medical student at Universidade Federal de Minas Gerais.

E-mail: bruno_campos[at]

Test question

(Medical Residence 2009 - UFF) Young patient, with history of jaundice and recurring pruritus, shows elevated transaminases over 5 times the normal range, gamma-GT and alkaline phosphatase over 1,5 times normal range, HAV serology negative, negative HBsAg, positive Anti-HBs, positive HBc, positive anti-HCV, hypergammaglobulinemia and positive Anti-LKM1. The most likely diagnosis is:

a) Acute hepatitis C


b) Chronic hepatitis B


c) Autoimmune hepatits


d) Primary sclerosing cholangitis


e) Primary billiar cholangitis



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