Primary sclerosing cholangitis
ICD-10 K83.0 · ICD-11 DB96.2

Treatment of Primary Sclerosing Cholangitis with Clinically Significant Portal Hypertension

Clinical Scenario

This protocol applies to patients with primary sclerosing cholangitis who have developed clinically significant portal hypertension (CSPH). CSPH is established by gastro-oesophageal varices on endoscopy, a hepatic venous pressure gradient (HVPG) of ≥10 mmHg, or pathognomonic imaging findings including portosystemic collaterals and ascites.

Treatment Approach

Management in this setting may involve a transjugular intrahepatic portosystemic shunt (TIPS), used either as a rescue intervention for refractory or uncontrollable variceal bleeding, or applied pre-emptively within 72 hours in high-risk acute presentations.

Full eligibility criteria, risk stratification thresholds, and the complete structured regimen are available in the protocol below.

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).

Transjugular intrahepatic portosystemic shunt (TIPS) can be performed as rescue TIPS in patients with refractory/uncontrollable variceal bleeding, or pre-emptive TIPS (<72 h) for acute variceal bleeding in high-risk patients (Child-Pugh class C patients, patients with Child-Pugh class B and active bleeding at endoscopy or HVPG ≥20 mmHg).

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