Treatment of Primary Sclerosing Cholangitis in Clinically Significant Portal Hypertension

In patients with primary sclerosing cholangitis who have developed clinically significant portal hypertension (CSPH), management must specifically address the elevated risk of portal hypertension-related decompensation alongside the underlying biliary disease.

Clinical scenario — CSPH: Clinically significant portal hypertension is established by the presence of gastro-oesophageal varices on endoscopy, a hepatic venous pressure gradient (HVPG) of ≥10 mmHg on invasive measurement, or pathognomonic imaging findings including portosystemic collaterals and ascites — provided that non-portal hypertensive causes such as malignancy have been excluded.

Management approach (partial overview)

Non-selective beta blockade is central to preventing portal hypertension-related decompensation; in a defined subset of patients, an endoscopic intervention may be the appropriate alternative. The complete patient-selection criteria, decision algorithm, and full regimen are available in the structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jhep.2022.05.011

Clinically significant portal hypertension (CSPH) is defined by either endoscopic finding of gastro-oesophageal varices (GEVs), invasive measurement of hepatic venous pressure gradient (HVPG) ≥10 mmHg, or pathognomonic imaging findings including portosystemic collaterals and ascites (given that non-portal hypertensive conditions including malignancy have been excluded).
Patients with portal hypertension should be treated with non-selective beta blockers (NSBBs) to prevent portal hypertension-related decompensation.
In compensated patients with high-risk varices, who have contraindications or intolerance to NSBBs, endoscopic band ligation (EBL) is recommended to prevent first variceal bleeding.

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