When Primary sclerosing cholangitis is complicated by clinically significant portal hypertension (CSPH), the clinical priorities shift substantially. This protocol defines the specific sub-population and the initial therapeutic strategy indicated in this setting.
Clinically significant portal hypertension is established by the presence of gastro-oesophageal varices on endoscopy, a hepatic venous pressure gradient (HVPG) of ≥10 mmHg, or pathognomonic imaging findings — including portosystemic collaterals and ascites — once non-portal hypertensive causes such as malignancy have been excluded.
The first-line strategy in this setting brings together a vasoactive agent and an endoscopic intervention — forming the recommended initial approach for acute variceal bleeding in this population. Specific agent selection, sequencing, and further management steps are defined in the full protocol.
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).
The combination of vasoactive drugs and EBL is recommended as the first therapeutic option for acute variceal bleeding.
DOI: 10.1016/j.jhep.2022.05.011
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