Читать книгу Pathy's Principles and Practice of Geriatric Medicine - Группа авторов - Страница 469

Primary biliary cholangitis (PBC), previously called primary biliary cirrhosis, is an autoimmune disease where T‐cells attack the small liver bile ducts and gradually cause destruction. It is a relatively rare disease with a prevalence of 1:50,000 and occurs primarily in women (90%), usually starting between the ages of 30 and 65. 60% of patients are asymptomatic at presentation, and the disease is suspected on inspection of liver enzymes showing elevation of alkaline phosphatase and sometimes bilirubin. 40% present with symptoms of fatigue and pruritus, and some patients present with clinical manifestations of cirrhosis. Physical signs include hyperpigmentation, jaundice, xanthomas, xerosis (dry skin), dermatographism, fungal infections of the feet or nails, and enlargement of the spleen in the absence of cirrhosis due to non‐portal hypertension. Like most autoimmune diseases, PBC is associated with other autoimmune diseases, especially Sjögren's syndrome, thyroid disease, scleroderma, and rheumatoid arthritis.

The disease should be looked for in any patient with elevated alkaline phosphatase, which is liver derived; this can be ascertained by fractionating the alkaline phosphatase or getting a gamma‐glutyl‐transferase (GGT) level that is also elevated. An ultrasound should be done to rule out an obstructive cause for the abnormality. If alkaline phosphatase is at least 1.5 times the upper limit of normal, blood tests should be done for antimitochondrial antibodies (AMA), or sp100 or gp210 antibodies if AMA is absent. A liver biopsy is often helpful, as it can show destruction of interlobular bile ducts and is relatively specific. The prognosis of this disease is usually good, and progress of the disease can be slowed with treatment with ursodeoxycholic acid or, if this does not lower alkaline phosphatase, with fenofibrate and obeticholic acid.

It is important for the geriatric physician to recognize this disease, as early treatment prevents end‐stage liver disease, patients can present with variceal bleeding in the absence of cirrhosis, and osteoporosis is more prevalent in PBC.²⁵