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Reliable, easy-to-read patient information on common medical conditions β€” written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Portal hypertension causes

Introduction

Overview

Overview

Life-threatening causes of portal hypertension include cirrhosis, severe portal venous obstruction or thrombosis (Budd-Chiari syndrome), and fulminant hepatic failure (e.g., due to hepatitis). Common causes for portal hypertension include alcoholic hepatitis, autoimmune disease, bacterial intestinal infections (e.g., recurrent E.coli infection), chronic hepatitis, cirrhosis, fatty liver, schistosomiasis, and sickle cell disease.

Causes

Causes

Life-threatening Causes

Common Causes

Portal hypertension may be caused by:[1][2][3]

Less Common Causes

Less common causes of portal hypertension include:[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]

Genetic Causes

Portal hypertension is caused by a mutation in the following genes:[21][22][23][24][25][26][21][27][28][29][30]

Causes classified by etiology

Prehepatic

Prehepatic causes of portal hypertension include:[2][15][16]

Intrahepatic

Intrahepatic causes of portal hypertension include:[1][5][6][7][8][9][10][11][12][13][14][17][18][19][20]

Posthepatic

Posthepatic causes of portal hypertension include:[3][4]

Causes by Organ System

Cardiovascular Cardiomyopathy, Congestive heart failure, Constrictive pericarditis, Inferior vena cava obstruction, Splanchnic arteriovenous fistula, Tricuspid insufficiency.
Chemical/Poisoning Arsenicals.
Dermatological NISCH syndrome.
Drug Side Effect Didanosine, Thioguanine, Vitamin A, Azathioprine, Cis-thioguanine, Antiretroviral therapy, Adams-Olivier syndrome.
Ear Nose Throat No underlying causes
Endocrine Hashimoto’s thyroiditis.
Environmental Vitamin A, Alcoholic hepatitis.
Gastroenterology Alcoholic hepatitis, Biliary atresia, Budd-Chiari syndrome, Cholestasis, Chronic hepatitis, Chronic liver disease, Cirrhosis, Congenital atresia or stenosis of portal vein, Congenital hepatic fibrosis, Congenital narrowing of the portal vein, Cruveilhier-Baumgarten syndrome, Fatty liver, Focal nodular hyperplasia, Fulminant hepatic failure, Hepatic amyloidosis with intrahepatic cholestasis, Hepatic arterioportal fistula, Hepatic portal vein obstruction, Hepatic vein occlusion, Hepatic vein thrombosis, Hepatic venoocclusive disease with immunodeficiency, Idiopathic liver cirrhosis, Idiopathic portal hypertension, Idiopathic tropical splenomegaly, Liver fibrosis, Mosse syndrome, Neonatal hepatitis, Nodular regenerative hyperplasia of the liver, Obliterative portal venopathy, Partial nodular transformation, Portal hypertension due to intrahepatic block, Portal vein abnormality, Portal vein compression, Portal vein occlusion, Portal vein thrombosis , Primary biliary cirrhosis, Primary sclerosing cholangitis, Splenic vein thrombosis, Alpha-1-antitrypsin deficiency, Progressive familial intrahepatic cholestasis, Berry aneurysm, Hepatic metastasis, Pancreatic cancer, NISCH syndrome, Crohn’s disease.
Genetic Alpha-1-antitrypsin deficiency, COACH syndrome, Cystic fibrosis, Gaucher disease, Glycosylphosphatidylinositol deficiency, Hemochromatosis, Interferon gamma receptor 1 deficiency, NISCH syndrome, Progressive familial intrahepatic cholestasis, Wilson’s Disease , Hepatic venoocclusive disease with immunodeficiency, Turner syndrome, Leukotriene B4 (LTB4) mutation, Ectonucleoside triphosphate diphosphohydrolase 4 (ENTPD4) mutation, Endothelin receptor type A (EDNRA) mutation, Deoxyguanosine kinase (DGUOK) mutation, Adenosine deaminase (ADA) mutation, Phospholipase A2 (PL2G10) mutation, Cytochrome P450, family 4, subfamily F, polypeptide 3 (CYP4F3) mutation, Glutathione peroxidase 3 (GPX3) mutation, Prostaglandin E receptor 2 (PTGER2) mutation, Endothelin (EDN1) mutation, Natriuretic peptide receptor 3 (NPR3) mutation, Cluster of differentiation 44 (CD44) mutation, Transforming growth factor (TGF)-Ξ² mutation, ATP-binding cassette, subfamily C, member 1 (ABCC1) mutation, Phosphomannose isomerase deficiency
Hematologic Blackfan Diamond anemia, Congenital pure red cell aplasia , Myeloproliferative diseases, Mosse syndrome, Hemochromatosis, Osteomyelosclerosis, Hodgkin’s lymphoma, Sickle cell disease, Inherited thrombophilias, Antiphospholipid syndrome, Human immunodeficiency virus (HIV) infection, Myeloproliferative neoplasm, Common variable immunodeficiency syndrome.
Iatrogenic Solid organ transplant, Renal transplantation, Liver transplantation.
Infectious Disease Schistosomiasis , Umbilical sepsis, Viral hepatitis, Recurrent E.coli infection.
Musculoskeletal / Ortho Osteomyelosclerosis, Connective tissue diseases.
Neurologic Berry aneurysm, Cerebral calcification, Beck’s disease.
Nutritional / Metabolic Gaucher disease, Glycosylphosphatidylinositol deficiency, Wilson’s disease, Vitamin A, Alcoholic hepatitis.
Obstetric/Gynecologic Antiphospholipid syndrome.
Oncologic Extrinsic compression (tumors), Hepatic metastasis, Hodgkin’s lymphoma, Pancreatic cancer.
Opthalmologic No underlying causes
Overdose / Toxicity Didanosine, Thioguanine.
Psychiatric No underlying causes
Pulmonary Cystic fibrosis, Alpha-1-antitrypsin deficiency, Berry aneurysm, Cirrhosis, Pulmonary emphysema, Cerebral calcification.
Renal / Electrolyte Polycystic kidney disease.
Rheum / Immune / Allergy Hepatic venoocclusive disease with immunodeficiency, Granulomatous diseases (Sarcoidosis, Tuberculosis), Hashimoto’s thyroiditis.
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Granulomatous diseases (Sarcoidosis, Tuberculosis), Extrinsic compression (tumors), Inferior vena cava obstruction.

Causes in Alphabetical Order

References

References

  1. ↑ 1.0 1.1 Li X, Gao W, Chen J, Tang W (2000). “[Non-cirrhotic portal hypertension associated with autoimmune disease]”. Zhonghua Wai Ke Za Zhi (in Chinese). 38 (2): 101–3. PMIDΒ 11831999.
  2. ↑ 2.0 2.1 Kono K, Ohnishi K, Omata M, Saito M, Nakayama T, Hatano H, Nakajima Y, Sugita S, Okuda K (1988). “Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Escherichia coli in rabbits”. Gastroenterology. 94 (3): 787–96. PMIDΒ 3276575.
  3. ↑ 3.0 3.1 Kumar S, Joshi R, Jain AP (2007). “Portal hypertension associated with sickle cell disease”. Indian J Gastroenterol. 26 (2): 94. PMIDΒ 17558079.
  4. ↑ 4.0 4.1 Bayan K, TΓΌzΓΌn Y, Yilmaz S, Canoruc N, Dursun M (2009). “Analysis of inherited thrombophilic mutations and natural anticoagulant deficiency in patients with idiopathic portal hypertension”. J. Thromb. Thrombolysis. 28 (1): 57–62. doi:10.1007/s11239-008-0244-8. PMIDΒ 18685811.
  5. ↑ 5.0 5.1 Girard M, Amiel J, Fabre M, Pariente D, Lyonnet S, Jacquemin E (2005). “Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism?”. Am. J. Med. Genet. A. 135 (2): 186–9. doi:10.1002/ajmg.a.30724. PMIDΒ 15832360.
  6. ↑ 6.0 6.1 Maida I, Garcia-Gasco P, Sotgiu G, Rios MJ, Vispo ME, Martin-Carbonero L, Barreiro P, Mura MS, Babudieri S, Albertos S, Garcia-Samaniego J, Soriano V (2008). “Antiretroviral-associated portal hypertension: a new clinical condition? Prevalence, predictors and outcome”. Antivir. Ther. (Lond.). 13 (1): 103–7. PMIDΒ 18389904.
  7. ↑ 7.0 7.1 Nevens F, Fevery J, Van Steenbergen W, Sciot R, Desmet V, De Groote J (1990). “Arsenic and non-cirrhotic portal hypertension. A report of eight cases”. J. Hepatol. 11 (1): 80–5. PMIDΒ 2398270.
  8. ↑ 8.0 8.1 Fuss IJ, Friend J, Yang Z, He JP, Hooda L, Boyer J, Xi L, Raffeld M, Kleiner DE, Heller T, Strober W (2013). “Nodular regenerative hyperplasia in common variable immunodeficiency”. J. Clin. Immunol. 33 (4): 748–58. doi:10.1007/s10875-013-9873-6. PMCΒ 3731765. PMIDΒ 23420139.
  9. ↑ 9.0 9.1 Vaiphei K, Bhatia A, Sinha SK (2011). “Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tracts”. Indian J Pathol Microbiol. 54 (1): 25–31. doi:10.4103/0377-4929.77319. PMIDΒ 21393872.
  10. ↑ 10.0 10.1 De Boer NK, Tuynman H, Bloemena E, Westerga J, Van Der Peet DL, Mulder CJ, Cuesta MA, Meuwissen SG, Van Nieuwkerk CM, Van Bodegraven AA (2008). “Histopathology of liver biopsies from a thiopurine-naΓ―ve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia”. Scand. J. Gastroenterol. 43 (5): 604–8. doi:10.1080/00365520701800266. PMIDΒ 18415755.
  11. ↑ 11.0 11.1 Sarin SK, Mehra NK, Agarwal A, Malhotra V, Anand BS, Taneja V (1987). “Familial aggregation in noncirrhotic portal fibrosis: a report of four families”. Am. J. Gastroenterol. 82 (11): 1130–3. PMIDΒ 3499813.
  12. ↑ 12.0 12.1 Imai Y, Minami Y, Miyoshi S, Kawata S, Saito R, Noda S, Tamura S, Nishikawa M, Tajima K, Tarui S (1986). “Idiopathic portal hypertension associated with Hashimoto’s disease: report of three cases”. Am. J. Gastroenterol. 81 (9): 791–5. PMIDΒ 2944377.
  13. ↑ 13.0 13.1 Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, Turon F, Hernandez-Gea V, Bosch J, Garcia-PagΓ‘n JC (2014). “Idiopathic portal hypertension: natural history and long-term outcome”. Hepatology. 59 (6): 2276–85. doi:10.1002/hep.26904. PMIDΒ 24155091.
  14. ↑ 14.0 14.1 de Lonlay P, Seta N (2009). “The clinical spectrum of phosphomannose isomerase deficiency, with an evaluation of mannose treatment for CDG-Ib”. Biochim. Biophys. Acta. 1792 (9): 841–3. doi:10.1016/j.bbadis.2008.11.012. PMIDΒ 19101627.
  15. ↑ 15.0 15.1 Allison MC, Mowat A, McCruden EA, McGregor E, Burt AD, Briggs JD, Junor BJ, Follett EA, MacSween RN, Mills PR (1992). “The spectrum of chronic liver disease in renal transplant recipients”. Q. J. Med. 83 (301): 355–67. PMIDΒ 1438671.
  16. ↑ 16.0 16.1 Gane E, Portmann B, Saxena R, Wong P, Ramage J, Williams R (1994). “Nodular regenerative hyperplasia of the liver graft after liver transplantation”. Hepatology. 20 (1 Pt 1): 88–94. PMIDΒ 8020909.
  17. ↑ 17.0 17.1 Vernier-Massouille G, Cosnes J, Lemann M, Marteau P, Reinisch W, Laharie D, Cadiot G, Bouhnik Y, De Vos M, Boureille A, Duclos B, Seksik P, Mary JY, Colombel JF (2007). “Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine”. Gut. 56 (10): 1404–9. doi:10.1136/gut.2006.114363. PMCΒ 2000290. PMIDΒ 17504943.
  18. ↑ 18.0 18.1 Calabrese E, Hanauer SB (2011). “Assessment of non-cirrhotic portal hypertension associated with thiopurine therapy in inflammatory bowel disease”. J Crohns Colitis. 5 (1): 48–53. doi:10.1016/j.crohns.2010.08.007. PMIDΒ 21272804.
  19. ↑ 19.0 19.1 Roulot D (2013). “Liver involvement in Turner syndrome”. Liver Int. 33 (1): 24–30. doi:10.1111/liv.12007. PMIDΒ 23121401.
  20. ↑ 20.0 20.1 Geubel AP, De Galocsy C, Alves N, Rahier J, Dive C (1991). “Liver damage caused by therapeutic vitamin A administration: estimate of dose-related toxicity in 41 cases”. Gastroenterology. 100 (6): 1701–9. PMIDΒ 2019375.
  21. ↑ 21.0 21.1 Campia U, Cardillo C, Panza JA (2004). “Ethnic differences in the vasoconstrictor activity of endogenous endothelin-1 in hypertensive patients”. Circulation. 109 (25): 3191–5. doi:10.1161/01.CIR.0000130590.24107.D3. PMIDΒ 15148269.
  22. ↑ Mandel H, Szargel R, Labay V, Elpeleg O, Saada A, Shalata A, Anbinder Y, Berkowitz D, Hartman C, Barak M, Eriksson S, Cohen N (2001). “The deoxyguanosine kinase gene is mutated in individuals with depleted hepatocerebral mitochondrial DNA”. Nat. Genet. 29 (3): 337–41. doi:10.1038/ng746. PMIDΒ 11687800.
  23. ↑ Blackburn MR, Lee CG, Young HW, Zhu Z, Chunn JL, Kang MJ, Banerjee SK, Elias JA (2003). “Adenosine mediates IL-13-induced inflammation and remodeling in the lung and interacts in an IL-13-adenosine amplification pathway”. J. Clin. Invest. 112 (3): 332–44. doi:10.1172/JCI16815. PMCΒ 166289. PMIDΒ 12897202.
  24. ↑ Kotani, Kohei; Kawabe, Joji; Morikawa, Hiroyasu; Akahoshi, Tomohiko; Hashizume, Makoto; Shiomi, Susumu (2015). “Comprehensive Screening of Gene Function and Networks by DNA Microarray Analysis in Japanese Patients with Idiopathic Portal Hypertension”. Mediators of Inflammation. 2015: 1–10. doi:10.1155/2015/349215. ISSNΒ 0962-9351.
  25. ↑ Chu FF, Esworthy RS, Doroshow JH, Doan K, Liu XF (1992). “Expression of plasma glutathione peroxidase in human liver in addition to kidney, heart, lung, and breast in humans and rodents”. Blood. 79 (12): 3233–8. PMIDΒ 1339300.
  26. ↑ Yokomizo T, Izumi T, Chang K, Takuwa Y, Shimizu T (1997). “A G-protein-coupled receptor for leukotriene B4 that mediates chemotaxis”. Nature. 387 (6633): 620–4. doi:10.1038/42506. PMIDΒ 9177352.
  27. ↑ Lopez MJ, Wong SK, Kishimoto I, Dubois S, Mach V, Friesen J, Garbers DL, Beuve A (1995). “Salt-resistant hypertension in mice lacking the guanylyl cyclase-A receptor for atrial natriuretic peptide”. Nature. 378 (6552): 65–8. doi:10.1038/378065a0. PMIDΒ 7477288.
  28. ↑ Aruffo A, Stamenkovic I, Melnick M, Underhill CB, Seed B (1990). “CD44 is the principal cell surface receptor for hyaluronate”. Cell. 61 (7): 1303–13. PMIDΒ 1694723.
  29. ↑ Derynck R, Akhurst RJ, Balmain A (2001). “TGF-beta signaling in tumor suppression and cancer progression”. Nat. Genet. 29 (2): 117–29. doi:10.1038/ng1001-117. PMIDΒ 11586292.
  30. ↑ Cole SP, Bhardwaj G, Gerlach JH, Mackie JE, Grant CE, Almquist KC, Stewart AJ, Kurz EU, Duncan AM, Deeley RG (1992). “Overexpression of a transporter gene in a multidrug-resistant human lung cancer cell line”. Science. 258 (5088): 1650–4. PMIDΒ 1360704.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Eiman Ghaffarpasand, M.D. [2]