A 45-year-old woman recently diagnosed with Sjögren's syndrome complains of severe pruritus that slowly progressed over the last year. She says that pruritus is especially prominent over palms and soles and is unbearable during the night. Laboratory testing shows: This patient most likely has which of the following conditions?

Question

A 45-year-old woman recently diagnosed with Sjögren's syndrome complains of severe pruritus that slowly progressed over the last year.  She says that pruritus is especially prominent over palms and soles and is unbearable during the night.  Laboratory testing shows:  This patient most likely has which of the following conditions?

Quizplus · Accepted Answer

11ec160b_395d_9b7e_9249_412f81a21eb2_MD0001_00 This patient is suffering from primary biliary cholangitis (PBC), a chronic liver disease characterized by autoimmune destruction of the intrahepatic bile ducts and cholestasis. The condition presents most commonly in middle-aged women and is insidious in onset. Pruritus is usually the first symptom and may be very severe, especially at night. Fatigue is also often reported. Physical findings typically include hepatosplenomegaly and xanthomatous lesions in the eyelids or in the skin and tendons. As the disease progresses, jaundice, steatorrhea, and portal hypertension may develop. Abnormalities on laboratory testing are consistent with cholestasis and include elevated alkaline phosphatase cholesterol, and bile acids (causing pruritus) due to spillage of bile into the blood. Diagnosis is confirmed with demonstration of anti-mitochondrial antibodies in the serum. Associated conditions include autoimmune diseases such as Sjögren's syndrome, Raynaud's syndrome, systemic sclerosis, and Hashimoto's thyroiditis. (Choices A and B) Expected laboratory findings in a patient with acute viral hepatitis include significant elevations in ALT and AST (with ALT > AST), followed by rises in bilirubin. (Choice C) Alcohol-induced hepatitis is typically associated with a significant drinking history and is commonly characterized by an AST:ALT ratio greater than 2:1. (Choice D) Budd-Chiari syndrome arises secondary to thrombotic occlusion of the hepatic veins and/or the intra- or suprahepatic inferior vena cava. The venous occlusion causes the sinusoidal pressure to increase, ultimately leading to portal hypertension, ascites, hepatomegaly, and splenomegaly. (Choices F and G) Hemochromatosis and Wilson's disease cause hepatocellular injury and thus are associated with elevated ALT and AST rather than cholestatic picture. Educational objective: Primary biliary cholangitis is a chronic liver disease characterized by autoimmune destruction of the intrahepatic bile ducts and cholestasis (elevated alkaline phosphatase). The condition is most common in middle-aged women, with severe pruritus (especially at night) often one of the first reported symptoms.

A 45-Year-Old Woman Recently Diagnosed with Sjögren's Syndrome Complains of Severe