Patients with PSC may be asymptomatic and diagnosed as part of the evaluation of abnormal laboratory tests, or they may have symptoms such as fatigue and pruritus. Physical examination may reveal jaundice, hepatomegaly, splenomegaly, and excoriations, though it is often normal. Liver biochemical tests usually demonstrate a cholestatic pattern, with elevation of the serum alkaline phosphatase predominating in most patients. Radiographic findings include abnormal appearing bile ducts with wall thickening, dilations, and strictures.
●Diagnosis
PSC should be considered in patients with a cholestatic pattern of liver test abnormalities (particularly an elevated alkaline phosphatase), especially those with underlying inflammatory bowel disease. The diagnosis is then made by showing cholangiographic evidence of characteristic bile duct changes (multifocal strictures, segmental dilations) and excluding secondary causes of sclerosing cholangitis. A percutaneous liver biopsy may support the diagnosis of PSC, but it is rarely diagnostic. In patients with characteristic findings on cholangiography, a liver biopsy is typically not required. However, liver biopsy is required for patients with suspected small duct PSC or if other conditions such as an overlap syndrome with autoimmune hepatitis are suspected.
●Differential diagnosis
PSC needs to be differentiated from secondary causes of sclerosing cholangitis and IgG4-associated cholangitis/autoimmune pancreatitis. In addition, PSC autoimmune hepatitis overlap syndrome should be considered.
