Yeungnam Univ J Med.  2018 Jun;35(1):89-93. 10.12701/yujm.2018.35.1.89.

Non-cirrhotic portal hypertension in an ankylosing spondylitis patient

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center, Busan, Korea. eyerheu1@nate.com

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.

Keyword

Portal hypertension; Ankylosing spondylitis; Perisplenic varix

MeSH Terms

Arsenic
Arthritis, Rheumatoid
Autoimmune Diseases
Biopsy
Esophageal and Gastric Varices
Fibrosis
Hepatic Veins
Humans
Hypertension, Portal*
Liver
Liver Cirrhosis
Lupus Erythematosus, Systemic
Portal Pressure
Scleroderma, Systemic
Spondylitis, Ankylosing*
Varicose Veins
Vinyl Chloride
Arsenic
Vinyl Chloride
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