Journal of Yeungnam Medical Science

Yeungnam University College of Medicine, Yeungnam University Institute Medical Science (영남대 의대 학술지 편집위원회)
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Non-cirrhotic portal hypertension in an ankylosing spondylitis patient

  • Park, Sukki (Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center) ;
  • Lee, Ji Hyun (Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center) ;
  • Choi, Joon Sul (Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center) ;
  • Kim, Hyun Woo (Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center) ;
  • Shim, Beom Jin (Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center) ;
  • Choi, Won Kyu (Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center) ;
  • Kim, Sang Hyun (Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center)
  • Received : 2016.07.08
  • Accepted : 2016.11.17
  • Published : 2018.06.30
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Abstract

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.

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References

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