What is the recommended management of non‑cirrhotic portal fibrosis (idiopathic portal hypertension)?

Management of Non-Cirrhotic Portal Fibrosis (Idiopathic Portal Hypertension)

Manage non-cirrhotic portal fibrosis (NCPF/INCPH) using the same approach as cirrhotic portal hypertension: endoscopic band ligation plus non-selective beta-blockers for variceal bleeding, with TIPS reserved for refractory cases, and covered stents preferred when TIPS is performed. 1

Initial Diagnostic Confirmation

Before initiating treatment, confirm the diagnosis requires three elements 2, 3:

• Liver biopsy demonstrating absence of cirrhosis with characteristic findings of portal fibrosis, hepatoportal sclerosis, or nodular regenerative hyperplasia 2, 4
• Patent portal and hepatic veins on Doppler ultrasound to exclude thrombosis 2, 5
• Exclusion of other chronic liver diseases including viral hepatitis, NASH, autoimmune hepatitis, hemochromatosis, Wilson disease, and schistosomiasis 2

Screen for prothrombotic disorders in all patients, as approximately 40% harbor thrombophilic conditions 2. This includes testing for myeloproliferative neoplasms, inherited thrombophilias, and antiphospholipid syndrome 3.

Management of Variceal Bleeding

Acute Bleeding Episode

• Initiate vasoactive drugs immediately (somatostatin/octreotide or terlipressin) when variceal bleeding is suspected 2
• Transfuse red blood cells conservatively: start at hemoglobin 7 g/dL with target 7-9 g/dL, as excessive transfusion paradoxically increases portal pressure 2
• Perform endoscopic band ligation as first-line therapy—this is superior to sclerotherapy 2, 5, 4
• Never use tranexamic acid in active variceal bleeding as it increases thrombotic risk 2

Prevention of Rebleeding (Secondary Prophylaxis)

• Combine non-selective beta-blockers (NSBBs) with endoscopic band ligation for optimal prevention of rebleeding 2, 5
• NSBBs reduce portal pressure by decreasing cardiac output and causing splanchnic vasoconstriction 2, 5
• Target a hepatic venous pressure gradient reduction of 10-12% or to <12 mmHg 5

The combination approach is critical here—while endoscopic therapy addresses the varices directly, NSBBs provide systemic portal pressure reduction 2.

TIPS for Refractory Cases

TIPS should be considered when endoscopic and pharmacological therapy fail to control bleeding 1, 5. The 2020 Gut guidelines and 2022 North American recommendations both support this approach 1.

TIPS-Specific Considerations for NCPF

• Use covered stents as they are preferred over bare metal stents 1
• Apply the same selection criteria as cirrhosis, but pay particular attention to hepatic encephalopathy risk factors 1
• TIPS demonstrates good clinical outcomes in NCPF with 5-year survival of 60-89% and effective ascites control 1, 5
• However, hepatic encephalopathy rates exceed 35% even in NCPF patients despite preserved liver function 1, 2, 5

Pre-TIPS Screening

Screen all elective TIPS candidates for covert and overt encephalopathy using at least two of the following 1:

• Psychometric hepatic encephalopathy score (PHES) testing
• Stroop testing
• Critical Flicker Frequency
• Spectral Enhanced or quantitative EEG

Covert hepatic encephalopathy is a relative contraindication to elective TIPS 1.

Portal Vein Thrombosis Management

Screen for portal vein thrombosis at least every 6 months as NCPF has higher PVT incidence than cirrhosis 2, 5.

Anticoagulation Strategy

• Initiate immediate anticoagulation for acute PVT, which achieves recanalization in 54% of cases 2, 5
• Long-term anticoagulation is mandatory for patients with underlying myeloproliferative neoplasms 2
• Consider permanent anticoagulation for those with strong prothrombotic conditions or history of recurrent thrombosis 2

The decision to anticoagulate must balance thrombotic risk against bleeding risk from varices, but in NCPF with preserved liver function, anticoagulation is generally better tolerated than in cirrhosis 3, 6.

Management of Other Complications

Portal Hypertensive Gastropathy

• Treat bleeding with portal pressure-lowering measures (NSBBs) rather than hemostatic correction 2, 5
• Consider TIPS for refractory bleeding when medical therapy fails 5

Refractory Ascites

TIPS may be considered for selected patients with refractory ascites, though this is less common in NCPF than cirrhosis 1, 7.

Liver Transplantation

Consider liver transplantation only for unmanageable portal hypertension complications or progressive liver failure 2, 5. This is rarely needed in NCPF since liver synthetic function is typically preserved, unlike cirrhosis 5, 4.

Key Clinical Pitfalls

Do not avoid TIPS in NCPF due to concerns about technical feasibility—TIPS is technically feasible and effective in NCPF with outcomes similar to or better than cirrhosis 1, 5. The main concern is the surprisingly high encephalopathy rate despite preserved liver function 1, 2.

Do not overlook prothrombotic screening—this is more critical in NCPF than cirrhosis and directly impacts anticoagulation decisions 2, 3.

Do not undertransfuse or overtransfuse during acute bleeding—the 7-9 g/dL target is evidence-based, as higher targets worsen portal pressure 2.