In all patients with a new diagnosis of PSC and no previous history or symptoms of inflammatory bowel disease, a full colonoscopy with biopsies should be performed. Colon cancer risk is significantly increased in patients with both PSC and ulcerative or Crohn colitis. In such patients surveillance colonoscopy with biopsies should be carried out at one- to two-year intervals.
The term hepatic osteodystrophy encompasses both osteopenia (T score between 1 and 2.5) and osteoporosis (T scores beneath 2.5). The incidence of osteoporosis in PSC ranges between 4% and 10%. The incidence increases with age and with increasing duration of disease. Accordingly, hepatic osteodystrophy should be checked by bone density examinations to exclude osteopenia or osteoporosis in all newly diagnosed patients with PSC. Although no data are available on subsequent appropriate intervals it appears reasonable to screen for osteopenia at 2–3 year intervals after an initial evaluation.
PSC is a progressive, debilitating disease for which few treatment options are currently available. Although many classes of drugs have been studied, none has shown efficacy in slowing disease progression. A major focus of medical treatment, therefore, is on managing symptoms.
Pruritus is common in PSC patients and is often difficult to control; this is especially vexing when it causes nocturnal symptoms and interferes with sleep. Medications that have proved useful in some patients include bile salt–sequestering agents such as cholestyramine, as well as naltrexone and rifampin. One agent that may be helpful in promoting better sleep is doxepin.
Ursodeoxycholic acid (UDCA) is the most extensively evaluated treatment in patients with PSC. Doses of 10–15 mg/ kg/day have not shown benefit, while doses of 20–30 mg/ kg/day have shown modest improvement in liver biochemical tests. However, such treatment has not resulted in survival benefit or delay in the need for liver transplantation. The latest AASLD (American Association for the Study of Liver Disease) guidelines do not recommend UDCA for medical therapy.
Other agents that have been studied include corticosteroids, budesonide, cyclosporine, methotrexate, azathioprine and 6-mercaptopurine, penicillamine, tacrolimus, pentoxifylline, colchicine, cholestyramine, mycophenolate, and anti-TNF monoclonal antibodies. None of these therapies has consistently been shown to halt disease progression.
In patients with increases in serum bilirubin and/or worsening pruritus or progressive bile duct dilatation, it is appropriate to perform an ERCP to exclude a proximal dominant stricture. In patients with a dominant stricture, management with endoscopic dilatation with or without stenting is usually recommended. The percutaneous approach is reserved for patients with a proximal dominant stricture in whom an endoscopic approach failed. Importantly, brush cytology and/or endoscopic biopsy should be performed to exclude a superimposed malignancy prior to endoscopic therapy for a dominant stricture.
Immunoglobulin G4–associated cholangitis (IAC) can mimic PSC. Approximately 60% of IAC patients treated with corticosteroids have resolution of strictures and normalization of liver enzymes. Thus, this seeming variant of PSC appears to be responsive to corticosteroids (see Chapter 27 for details).
Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology
Ghazale A, Chari ST, Zang L, et al. Immunoglobulin G4
-associated cholangitis: clinical profile and response to therapy. Gastroenterology.
Smith T, Befeler AS. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Curr Gastroenterol Rep.
Liver transplantation is best choice for patients with advanced PSC; 5-year survival rates of approximately 85% and a 10-year survival rate of approximately 70% have been reported.