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Ǵм Yeungnam Univ J Med 2018;35(1):89-93


Non-cirrhotic portal hypertension in an ankylosing spondylitis patient

Sukki Park, Ji Hyun Lee, Joon Sul Choi, Hyun Woo Kim, Beom Jin Shim, Won Kyu Choi, Sang Hyun Kim
Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center, Busan, Korea
Corresponding Author: Ji Hyun Lee, Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center, 121, Junggu-ro, Jung-gu, Busan 48972, Korea
Tel: +82-51-461-2732, Fax: +82-51-441-6950
E-mail: eyerheu1@nate.com

Received: July 8, 2016, Revised: October 29, 2016 Accepted: November 17, 2016


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.

Key Words: Keywords: Portal hypertension; Ankylosing spondylitis; Perisplenic varix