Portal hypertension causes

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Overview

Many conditions can result in portal hypertension. In North America and Europe, it is usually the result of an intrahepatic block due to cirrhosis of the liver. However, in less industrialized parts of the world, climate permitting, the major cause is schistosomiasis.

Causes

Common variable immunodeficiency syndrome[1]
Connective tissue diseases[2]
Crohn’s disease[3]
Solid organ transplant
•• Renal transplantation[4]
•• Liver transplantation[5]
Hashimoto's thyroiditis[6]
Autoimmune disease[7] |B02=• Bacterial intestinal infections
• Recurrent E.coli infection[8]
Human immunodeficiency virus (HIV) infection[9]
Antiretroviral therapy[10]|B03=• Thiopurine derivatives
•• Didanosine
•• Azathioprine[11]
•• Cis-thioguanine[12]
Arsenicals[13]
Vitamin A[14]|B04=• Adams-Olivier syndrome[15]
Turner syndrome[16]
• Phosphomannose isomerase deficiency[17]
• Familial cases[18] |B05=• Inherited thrombophilias [19]
Myeloproliferative neoplasm[19]
Antiphospholipid syndrome[19]
Sickle cell disease[20]


Prehepatic

Intrahepatic

Posthepatic

Causes by Organ System

Cardiovascular Cardiomyopathy, Congestive heart failure, Constructive pericarditis, Inferior vena cava obstruction, Splanchnic arteriovenous fistula, Tricuspid insufficiency
Chemical / poisoning No underlying causes
Dermatologic NISCH syndrome
Drug Side Effect Didanosine, Thioguanine
Ear Nose Throat No underlying causes
Endocrine No underlying causes
Environmental No underlying causes
Gastroenterologic 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
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
Hematologic Blackfan Diamond anemia, Congenital pure red cell aplasia , Myeloproliferative diseases, Mosse syndrome, Hemochromatosis, Osteomyelosclerosis, Hodgkin's lymphoma
Iatrogenic No underlying causes
Infectious Disease Schistosomiasis , Umbilical sepsis , Viral hepatitis
Musculoskeletal / Ortho Osteomyelosclerosis
Neurologic Berry aneurysm, Cerebral calcification
Nutritional / Metabolic Gaucher disease, Glycosylphosphatidylinositol deficiency, Wilson's disease
Obstetric/Gynecologic No underlying causes
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, and cerebral calcification
Renal / Electrolyte Polycystic kidney disease
Rheum / Immune / Allergy Hepatic venoocclusive disease with immunodeficiency, Granulomatous diseases (Sarcoidosis, Tuberculosis)]
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Granulomatous diseases (Sarcoidosis, Tuberculosis), Extrinsic compression (tumors)

Causes in Alphabetical Order


References

  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. PMC 3731765. PMID 23420139.
  2. 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. PMID 21393872.
  3. 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-naïve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia". Scand. J. Gastroenterol. 43 (5): 604–8. doi:10.1080/00365520701800266. PMID 18415755.
  4. 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. PMID 1438671.
  5. 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. PMID 8020909.
  6. 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. PMID 2944377.
  7. 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. PMID 11831999.
  8. 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. PMID 3276575.
  9. Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, Turon F, Hernandez-Gea V, Bosch J, Garcia-Pagán JC (2014). "Idiopathic portal hypertension: natural history and long-term outcome". Hepatology. 59 (6): 2276–85. doi:10.1002/hep.26904. PMID 24155091.
  10. 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. PMID 18389904.
  11. 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. PMC 2000290. PMID 17504943.
  12. 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. PMID 21272804.
  13. 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. PMID 2398270.
  14. 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. PMID 2019375.
  15. 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. PMID 15832360.
  16. Roulot D (2013). "Liver involvement in Turner syndrome". Liver Int. 33 (1): 24–30. doi:10.1111/liv.12007. PMID 23121401.
  17. 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. PMID 19101627.
  18. 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. PMID 3499813.
  19. 19.0 19.1 19.2 Bayan K, Tüzün 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. PMID 18685811.
  20. Kumar S, Joshi R, Jain AP (2007). "Portal hypertension associated with sickle cell disease". Indian J Gastroenterol. 26 (2): 94. PMID 17558079.

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