Do not miss any of the new cases! Subscribe above to receive our newsletter! ↑↑

Do not miss the opportunity to download our app on the Google Play Store. Click here to enjoy it.

Previous

Case 124

Next


Click on the images above to zoom in

A 39-year-old male patient attended a general practitioner complaining of chronic dizziness, pruritus and asthenia for the past five years with cyclical waxing and waning of symptoms. He reported worsening of his condition 6 weeks before, with the development of jaundice, abdominal distension, constipation and coluria. He denied any alterations in the color of the stools. On examination, he presented with jaundice (+/4+), painful epigastric and hypocondriac palpation and a liver palpable 2.5 cm below the right costal margin. No abnormalities were found in an abdominal ultrasound performed 1 month before. Laboratorial data included increased alkaline phosphatase, gamma glutamyl transpeptidase (GGT) and bilirubin levels.

Based on the clinical history and images provided, which is the most likely diagnosis?

a) Wilson’s disease

25%

b) Primary biliary cirrosis

25%

c) Primary sclerosing cholangitis

25%

d) Cholangiocarcinoma

25%
   

Comments

- The primary sclerosing cholangitis is a chronic cholestatic disease that results in progressive obliteration of intrahepatic and extrahepatic ducts.

- It is a rare condition, and affects men twice as often as women, with the mean age at diagnosis of 40 years.

- The etiology remains unknown and multifactorial, with a probable autoimmune component.

- The clinical presentation usually consists of fatigue and pruritus, although most of the patients is asymptomatic at diagnosis.

- The diagnosis is made by demonstrating multifocal strictures and segmental dilatations of the intrahepatic and extrahepatic biliary tree, preferably by magnetic resonance cholangiopancreatography.

- The outlook is grim and liver transplantation is the only potentially curative therapy.

References

- Kowdley KV, et al. Clinical manifestations and diagnosis of primary sclerosing cholangitis. In: UpToDate, Basow, DS (Ed), UpToDate, Waltham, MA, 2013.

- Kowdley KV, et al.Treatment of primary sclerosing cholangitis. In: UpToDate, Basow, DS (Ed), UpToDate, Waltham, MA, 2013.

- Kowdley KV, et al.Epidemiology and pathogenesis of primary sclerosing cholangitis. In: UpToDate, Basow, DS (Ed), UpToDate, Waltham, MA, 2013.

-Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010 Feb; 51(2):660-78.

-Decock, S, McGee P, Hirschfield GM. Autoimmune liver disease for the non-specialist. Clinical Review. BMJ 2009; 339:b3305.

Authors

- Juliana da Cunha Pimentel Ulhôa - 6th year medical student of UFMG School of Medicine

E-mail: ju_ulhoa[at]hotmail.com

- Rebeca Maciel Bizzotto - 6th year medical student of UFMG School of Medicine

E-mail: rebekmaciel[at]hotmail.com

Supervisors

- Professor Luciana Diniz Silva, member of the Department of Internal Medicine (UFMG School of Medicine). Email: lucianadinizsilva[at]gmail.com

- Professor Unaí Tupinambás, member of the Department of Internal Medicine (UFMG School of Medicine). Email: unaitupi[at]gmail.com

Reviewers

Glauber Eliazar, Emília Valle and professor Viviane Parisotto

Translated by

Lucas Resende Lucinda, 6th year medical student of UFMG School of Medicine. 

E-mail: lucasresendebh[at]hotmail.com

Commentics

Sorry, there is a database connection problem.

Please check back again shortly.

Bookmark and Share




Unfortunately there is no english translation available yet for this case.

Please refer to the Portuguese version instead or come back later.





Follow us:      Twitter  |    Facebook  |    Get the news  |    E-mail