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

• Park S
• Lee JH
• Choi JS
• Kim HW
• Shim BJ
• Choi WK
• Kim SH

• KMID: 2415739
• DOI: http://doi.org/10.12701/yujm.2018.35.1.89

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

Figure

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  Fig. 1. Simple X-ray of sacroiliac joint, right (A), left (B), anteroposterior (C), and lateral radiographs (D) of the lumbosacral spine showed bilateral sacroiliitis and syndesmophytes of lumbar spines. Computed tomography image of the sacroiliac joints (E) showed erosions and eburnation (arrows).

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  Fig. 2. Abdominal computed tomography shows perisplenic varices (arrows).

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  Fig. 3. Histologic finding shows mild and focal chronic hepatitis and cholestasis without any features suggesting fibrosis or cirrhosis (H&E stain, ×200).

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