No treatment has yet been shown to slow the progression of PSC. Management is therefore built around four things: controlling symptoms and complications as they arise, relieving obstruction caused by dominant strictures, watching for the cancers PSC predisposes to, and referring for liver transplantation when the liver can no longer do its work.
Medical therapy
Many drugs have been tried, but none is approved to modify the course of PSC. Ursodeoxycholic acid (UDCA) is the most studied. It improves the liver biochemistry — it lowers alkaline phosphatase — but it has not been shown to reduce the risk of cholangiocarcinoma, the need for liver transplantation or the risk of death. High-dose UDCA (28–30 mg/kg/day) is not recommended, and UDCA is no longer used routinely in newly diagnosed PSC, but moderate-dose UDCA (15–20 mg/kg/day) can be given because it improves liver biochemistry, though it has not been shown to change transplant-free survival. Because there is no disease-modifying drug, care is directed at the consequences of the disease rather than at the disease itself.
Endoscopic treatment of dominant strictures
When a dominant stricture causes symptoms or obstructs the flow of bile, endoscopic retrograde cholangiopancreatography (ERCP) allows the stricture to be dilated with a balloon and, if necessary, held open with a stent, and it lets the endoscopist take brushings or biopsies to look for cholangiocarcinoma. ERCP carries its own risks, including pancreatitis and cholangitis, so it is reserved for strictures that matter clinically rather than used as a routine test. Episodes of bacterial cholangitis are treated with antibiotics.
Surveillance
Because cholangiocarcinoma can develop at any stage and may cause no specific symptoms, patients are followed with regular imaging of the biliary tree, usually by magnetic resonance cholangiopancreatography (MRCP), to look for a tumour or for a stricture that is changing. Annual ultrasound is used to examine the gallbladder. Colonoscopy is part of care. In patients with PSC but no known IBD, it is done when PSC is diagnosed and then repeated every 3–5 years if no colitis is found, so that IBD is not missed. In patients who have both PSC and IBD, in whom the colorectal cancer risk is highest, it is done at diagnosis and then annually.
Liver transplantation
Liver transplantation is the only curative treatment for PSC. It is considered when the disease reaches decompensated cirrhosis, or earlier for complications that cannot be controlled — intractable pruritus, recurrent cholangitis, or a cholangiocarcinoma that is still confined to the liver and within transplant criteria. Transplantation removes the diseased bile ducts, but PSC can recur in the transplanted liver, so patients continue to be followed after surgery.