Journal of Yeungnam Medical Science

영남대 의대 학술지 편집위원회 (Yeungnam University College of Medicine, Yeungnam University Institute Medical Science)
권호 표지
DOI QR코드

DOI QR Code

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)
  • 투고 : 2016.07.08
  • 심사 : 2016.11.17
  • 발행 : 2018.06.30
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

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.

키워드

참고문헌

  1. Schouten JN, Verheij J, Seijo S. Idiopathic non-cirrhotic portal hypertension: a review. Orphanet J Rare Dis 2015;10:67. https://doi.org/10.1186/s13023-015-0288-8
  2. Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, et al. Idiopathic portal hypertension: natural history and long-term outcome. Hepatology 2014;59:2276-85. https://doi.org/10.1002/hep.26904
  3. Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 2002;51:275-80. https://doi.org/10.1136/gut.51.2.275
  4. Cazals-Hatem D, Hillaire S, Rudler M, Plessier A, Paradis V, Condat B, et al. Obliterative portal venopathy: portal hypertension is not always present at diagnosis. J Hepatol 2011;54:455-61. https://doi.org/10.1016/j.jhep.2010.07.038
  5. Vaiphei K, Bhatia A, Sinha SK. Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tracts. Indian J Pathol Microbiol 2011;54:25-31. https://doi.org/10.4103/0377-4929.77319
  6. Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL. Idiopathic noncirrhotic portal hypertension. Hepatology 2011;54:1071-81. https://doi.org/10.1002/hep.24422
  7. Ziol M, Poirel H, Kountchou GN, Boyer O, Mohand D, Mouthon L, et al. Intrasinusoidal cytotoxic CD8+ T cells in nodular regenerative hyperplasia of the liver. Hum Pathol 2004;35:1241-51. https://doi.org/10.1016/j.humpath.2004.06.016
  8. Juanola X, Mateo L, Domenech P, Bas J, Contreras N, Nolla JM, et al. Prevalence of antiphospholipid antibodies in patients with ankylosing spondylitis. J Rheumatol 1995;22:1891-3.
  9. Ungprasert P, Srivali N, Kittanamongkolchai W. Risk of coronary artery disease in patients with ankylosing spondylitis: a systematic review and meta-analysis. Ann Transl Med 2015;3:51.
  10. Schouten JN, Nevens F, Hansen B, Laleman W, van den Born M, Komuta M, et al. Idiopathic noncirrhotic portal hypertension is associated with poor survival: results of a long-term cohort study. Aliment Pharmacol Ther 2012;35:1424-33. https://doi.org/10.1111/j.1365-2036.2012.05112.x