At morning report this week, our residents and faculty had a great discussion on non-cirrhotic etiologies for portal hypertension and ascites. There is a great review article from Hepatology in 2011 by Schouten, et al, “Idiopathic Noncirrhotic Portal Hypertension”, which describes the different etiologies and how to evaluate patients with portal hypertension not due to cirrhosis. Also includes an extensive table of noncirrhotic causes of portal hypertension. Key points below from the article:
- Worldwide, most common cause of noncirrhotic portal hypertension (NCPH) is schistosomiasis.
- In Western countries, more common causes include intrahepatic and extrahepatic etiologies such as Budd-Chiari, primary biliary cirrhosis, PSC, congenital hepatic fibrosis, portal vein thrombosis, sarcoidosis, lymphoma compressing the portal vein.
- However, if none of the diagnosis mentioned above is made for the patient, then consider idiopathic noncirrhotic portal hypertension (INCPH).
- Proposed pathophysiology of INCPH:
- Strong expression of inducible NO synthetase (iNOS) and endothelial NO synthetase (eNOS) in the sinus-lining of the cells leading to splenomegaly and increased splenic venous flow and portal hypertension
- Obliteration of the portal venous microcirculation leading to increased intrahepatic resistance
- Etiology of INCPH can be divided into 5 categories: chronic infection, exposure to toxins, thrombophilia, immunological disorders, and genetic disorders.
- Most common immunosupressive medications associated with INCPH are thiopurines such as azathioprine and 6-mercaptopurine
- Management: no current universal guidelines regarding GI bleed prophylaxis, anticoagulation for risk of thrombus, and liver transplantation with few case reports
Check out the article below!