Case history
Case history
A 50-year-old woman undergoing health screening is found to have a cholestatic pattern on her liver function test results. Her alkaline phosphatase and gamma-GT concentrations are elevated, although transaminases, bilirubin, and albumin concentrations are normal. On questioning she mentions that she had been getting increasingly tired over the last few years but felt that this was simply a result of her age and work pattern. She also describes occasional itch that feels as if it is deep underneath the skin and that is not associated with a rash. She herself had no other past medical history but had a family member who had autoimmune thyroid disease. Clinical exam reveals no abnormal findings other than excoriations related to itch and xanthelasmata around the eyes.
Other presentations
Although most patients with PBC present early in the disease process with abnormal liver biochemistry (with or without the symptoms of itch, fatigue, or abdominal pain), occasionally patients will present with advanced liver disease. In these patients, the clinical features of cirrhosis would be more prominent than the features of PBC. Possible features would include ascites, splenomegaly, skin thinning, weight loss, and variceal bleeding. Jaundice may be significantly more prominent than would be expected in people with cirrhosis of different etiologies. PBC can present from the 20s onward and there is increasing evidence to suggest that it is more severe in nature and less responsive to first-line therapy in younger patients.[2]
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