Figure 3: Image (left) shows a portal bile duct with lymphocytic infiltration of the epithelium and severe chronic inflammation in the surrounding tissue.
Figure 4: This image (below) reveals poorly formed granumlomas, with multiple epitheloid histocytes, within the portal triads and surrounding severe chronic inflammation.
As in this case, primary biliary cirrhosis will often manifest with nonspecific findings on ultrasonography and CT. Common imaging findings include signs of portal hypertension, including splenomegaly, ascites, and varices. Hepatomegaly is also often present, particularly in earlier phases of the disease. Enlarged upper abdominal lymph nodes, which can enhance, are also a common finding. 12 MRI can have improved specificity, particularly in later stages of the disease, manifesting a periportal halo sign. This sign is characterized by low T1 and T2 signal intensity in a periportal distribution without mass effect. The sign reflects periportal parenchymal loss adjacent to regenera- tive nodule formation. Aside from diagnostic utility, cross sectional imaging can also be used for screening for hep- tocellular carcinoma, as these patients a carry an increased risk (~5%). 13,14 The classic findings on liver biopsy of patients with PBC is asymmetric destruction of the bile ducts within the portal triads. 2 Patients are further classified into stages (i.e., stage one through four) based on the degree of destruction and the number of bile ducts involved. Stage 1 is inflamma- tion localized to the portal triads, while stage 4 represents end-stage liver disease. 2 In 2009, The American Association for the Study of Liver Diseases (AASLD) published recom- mendations for the diagnosis of PBC. 11 The diagnosis can be made if any two of the following three criteria are met: • Biochemical evidence of cholestasis based mainly on alkaline phosphatase elevation • Presence of AMA
pruritis in PBC is thought to be directly related to cholestasis, decreased bile excretion, and subsequent accumulation of bile in the plasma and tissues. Fatigue is the most consistent symptom of PBC, occurring in about 70%-80% of patients. 8 The exact etiology is still unknown, but peripheral muscle fatigablilty 9 and autonomic dysfunction 10 as mechanisms have been proposed. Less common symptoms may include Sicca Syndrome (dry eyes andmouth), cutaneous calcinosis, Raynaud’s phenomenon, and dysphagia, as PBC can be as- sociated with other autoimmune diseases such as Sjogren’s syndrome and scleroderma. 2 The physical exam is often normal in the asymptomatic patient. Skin hyperpigmenta- tion due to melanin deposition may be seen earlier in the course, while jaundice is a latemanifestation. Hepatomegaly is found in about 70% of patients, and splenomegaly may develop as the disease progresses. 2 The diagnosis of PBC should always be considered in the setting of cholestasis after the exclusion of other liver diseases. Most patients have a significantly elevated alkaline phosphatase withmilder elevations in the aminotransferas- es. 11 The degree of alkaline phosphatase elevations generally correlates with the severity of ductopenia and inflammation. As the disease progresses, a rise in serum bilirubin, along with a decrease in serum albumin and platelets, may signal the development of cirrhosis and portal hypertension. Se- rum cholesterol levels are oftenmarkedly elevated as well. 11 While anti-nuclear antibody (ANA) is only positive in ap- proximately 70%of patients with PBC12, anti-mitochondrial antibody (AMA) is found in nearly 95% of cases. 11
J La State Med Soc VOL 166 May/June 2014 131
Made with FlippingBook - Online catalogs