What is the appropriate work‑up and management for a patient with non‑cirrhotic portal fibrosis (idiopathic portal hypertension) presenting with portal hypertension and normal liver imaging/biopsy?

Non-Cirrhotic Portal Fibrosis: Work-up and Management

For patients with non-cirrhotic portal fibrosis presenting with portal hypertension, manage portal hypertension complications according to cirrhosis guidelines, with endoscopic therapy plus non-selective beta-blockers as first-line treatment for variceal bleeding, while screening for prothrombotic conditions and considering anticoagulation in high-risk cases. 1

Diagnostic Confirmation

Before initiating treatment, confirm the diagnosis by meeting all required criteria 1:

• Document portal hypertension signs: Splenomegaly with hypersplenism, esophageal varices, ascites, or portosystemic collaterals on imaging 1
• Exclude cirrhosis: Liver biopsy showing absence of cirrhosis is mandatory 1
• Rule out other causes: Exclude viral hepatitis B/C, NASH/alcoholic steatohepatitis, autoimmune hepatitis, hemochromatosis, Wilson's disease, PBC, congenital hepatic fibrosis, sarcoidosis, and schistosomiasis 1
• Confirm vascular patency: Doppler ultrasound demonstrating patent portal and hepatic veins 1

Additional Diagnostic Work-up

• Screen for prothrombotic conditions: Western patients show 40% prevalence of thrombophilic disorders 1
• Evaluate for underlying causes: Check for immunological disorders, HIV infection, and medication exposure (azathioprine, didanosine) 1
• Assess for portal biliopathy: Perform MR cholangiography if persistent cholestasis or biliary abnormalities are present 1

Management of Portal Hypertension Complications

Variceal Bleeding Management

Primary treatment approach 2:

• Endoscopic band ligation is preferable to sclerotherapy for acute variceal bleeding 2
• Non-selective beta-blockers (NSBBs) should be initiated for prevention of variceal bleeding 2
  • NSBBs reduce portal pressure by decreasing cardiac output and causing splanchnic vasoconstriction 2
  • A 10-12% reduction in hepatic venous pressure gradient protects against variceal bleeding 2

Acute bleeding protocol 2:

• Initiate vasoactive drugs (somatostatin/octreotide or terlipressin) immediately when variceal bleeding is suspected 2
• Transfuse red blood cells conservatively: start at hemoglobin 7 g/dL with goal of 7-9 g/dL, as excessive transfusion paradoxically increases portal pressure 2
• Do NOT use tranexamic acid—it is contraindicated and increases thrombotic risk 2

Secondary prophylaxis 2:

• Combination therapy with NSBBs plus endoscopic band ligation is recommended for prevention of rebleeding 2

Refractory Cases

TIPS placement 2:

• Consider for uncontrolled bleeding despite endoscopic and pharmacological therapy 2
• Good clinical outcomes with 5-year survival of 60-89% in NCPF patients 2
• Caution: Hepatic encephalopathy rates may exceed 35% after TIPS 2
• Also effective for refractory ascites 3

Portal Vein Thrombosis Surveillance and Treatment

Screening protocol 2:

• Portal vein thrombosis has higher incidence in NCPF compared to cirrhosis 2
• Screen at least every 6 months with Doppler ultrasound 2

Anticoagulation considerations 1, 2:

• Long-term anticoagulation is indicated for underlying myeloproliferative neoplasms 1
• Consider permanent anticoagulation for strong prothrombotic conditions or history suggesting intestinal ischemia/recurrent thrombosis 1
• Early anticoagulation leads to recanalization in 54% of patients with portal vein thrombosis 2
• Treat underlying prothrombotic conditions according to corresponding guidelines 1

Portal Hypertensive Gastropathy

• Treat with portal pressure-lowering measures (NSBBs) rather than hemostatic correction 2
• Avoid large-volume blood product transfusion as it paradoxically increases portal pressure and worsens bleeding 2

Advanced Therapy Considerations

Liver transplantation 2:

• Consider for patients who develop liver failure or unmanageable portal hypertension-related complications 2
• Should be evaluated for all patients with decompensated disease as definitive treatment 3

Key Clinical Pitfalls

Preserved liver function is characteristic 4, 5:

• Unlike cirrhosis, NCPF patients typically maintain preserved liver function 4
• Laboratory tests often show normal or near-normal values despite significant portal hypertension 5
• This better hepatic reserve explains lower mortality from variceal bleeding compared to cirrhosis 6

Ascites presentation differs 5:

• Ascites is rarely observed initially in NCPF 5
• When present, it typically occurs in the context of precipitating factors (such as after variceal bleeding) 7
• This contrasts with cirrhosis where ascites is the most common complication 3

Prognosis is generally favorable 7, 5:

• Survival is mainly limited by concomitant disorders rather than liver disease itself 7
• Long-term prognosis is better than cirrhosis when ascites is absent 5