It is a replacement bile acid considered to be less hepatotoxic than the biliary secretions that accumulate in cholestatic disease. Fortunately, it also has very few significant side effects. In select patients, methotrexate may provide some improvement, but side effects, including bone marrow suppression and pneumonitis, restrict use considerably. Bile acid sequestrants can also be used and may improve systemic complaints, such as pruritis. For those with end-stage disease, liver transplant is the only effective therapy.
Aeromedical considerations are relatively straightforward.
As primary sclerosing cholangitis is a slowly progressive disease, sudden incapacitations are unlikely. Instead, the slow development of liver failure, and subsequent effects, will probably eventually remove the pilot from flight status. Close supervision for the development of encephalopathy and esophageal varices is important. Poor night vision may develop from a deficiency in vitamin A, a fat-soluble vitamin, and the pilot should be provided with appropriate supplements. The issue of flying after organ transplant raises many additional concerns that will not be covered at this time.