WBR0671

Author	[[PageAuthor::Yazan Daaboul, M.D. (Reviewed by Serge Korjian)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Pathology, MainCategory::Pathophysiology
Sub Category	SubCategory::Gastrointestinal
Prompt	[[Prompt::A 52-year-old woman presents to the physician's office complaining of excessive fatigue and pruritis. Her past medical history is significant for hypertension well-controlled on lisinopril, dyslipidemia on simvastatin, and Hashimoto's hypothyroidism on levothyroxine replacement therapy. She is a heavy smoker but does not consume alcohol. Her blood pressure is 110/60 mm Hg, heart rate is 110/min, and temperature is 36.7 ᵒC (98 ᵒF). Physical examination is remarkable for jaundice and skin excoriations, xanthelesmas, and mild hepatomegaly. Work-up reveals elevated antimitochondrial antibodies. Which of the following pathological findings best characterizes the patient's primary condition?]]
Answer A	AnswerA::Concentric periductal fibrosis showing an "onion skin" appearance
Answer A Explanation	[[AnswerAExp::Primary sclerosing cholangitis is characterized by the presence of concentric periductal fibrosis showing an "onion skin" appearance. ERCP reveals a "beading" appearance of the biliary tracts due to the presence of strictures and dilatations in the bile duct.]]
Answer B	AnswerB::Asymmetric destruction of the bile ducts within the portal triad
Answer B Explanation	AnswerBExp::Primary biliary cirrhosis is characterized by the presence of asymmetric destruction of the bile ducts within the portal triad.
Answer C	AnswerC::Diffuse fibrosis and nodular regeneration in the liver
Answer C Explanation	[[AnswerCExp::Hepatic cirrhosis is characterized by the presence of diffuse fibrosis and nodular regeneration in the liver. In advanced cirrhosis, patients have a shrunken liver. Cirrhosis is the final result of primary biliary cirrhosis, but is not the primary disease in this patient.]]
Answer D	AnswerD::Ballooning degeneration with focal necrosis and presence of Mallory-Denk bodies within hepatocytes
Answer D Explanation	[[AnswerDExp::Alcoholic hepatitis, due to excessive chronic alcohol consumption, is characterized by ballooning degeneration with focal necrosis and presence of Mallory-Denk bodies (or Mallory bodies), which are hyaline material surrounded by neutrophils, within hepatocytes. Typically, patients with alcohol hepatitis have elevated levels of serum IgA.]]
Answer E	AnswerE::Large neoplastic cells with hyperchromasia and prominent pleomorphism
Answer E Explanation	AnswerEExp::Poorly differentiated hepatocellular carcinoma is characterized by the presence of large neoplastic cells with hyperchromatia and prominent pleomorphism.
Right Answer	RightAnswer::B
Explanation	[[Explanation::Primary biliary cirrhosis (PBC) is a slowly progressive autoimmune hepatic pathology. It is characterized by the presence of portal inflammation and intrahepatic bile duct destruction that is immune-mediated. Eventually, loss of bile ducts lead to diminished secretion of toxic compounds that are retained in the liver and ultimately cause hepatic failure. The prevalence of PBC differs from one region to another; it is most prevalent in Northern Europe. Several environmental factors have been hypothesized to be associated with PBC such as infectious etiologies (E. coli and N. aromaticivorans) and exposure to chemicals or pesticides. Genetic predisposition my also play a role. PBC may be associated with other autoimmune diseases, such as Hashimoto's hypothyroidism. It is a common disease among middle-aged female patients and very uncommon in childhood and early adulthood. Antimitochondrial antibodies are found in more than 90% of patients and may be elevated even prior to the onset of symptoms. Fatigue and pruritis are the most common clinical presentations, but it is noteworthy to mention that more than 50% of patients are asymptomatic. Physical examination findings of jaundice, xanthelesma, and hepatomegaly are classical, but may not always be present or may become apparent late during the disease progression. Early diagnosis and management are important for prognosis, which may be very poor if not treated. Complications of PBC include: osteoporosis, hyperlipidemia, portal hypertension, annd fat-soluble vitamin deficiency. Treatment options include ursodeoxycholic acid, colchicine, and methotrexate. The only effective treatment, however, is liver transplantation with recurrence rates reaching 15% within 3 years and 30% within 10 years. Educational Objective: Primary biliary cirrhosis is characterized by the presence of asymmetric destruction of the interlobular bile ducts. References: Marshall KM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005; 353:1261-1273.]]
Approved	Approved::No
Keyword	WBRKeyword::Primary biliary cirrhosis, WBRKeyword::PBC, WBRKeyword::Autoimmune disorders, WBRKeyword::Autoimmune diseases, WBRKeyword::Autoimmune, WBRKeyword::Hepatic, WBRKeyword::Liver, WBRKeyword::Liver cirrhosis, WBRKeyword::Cirrhosis, WBRKeyword::Antimitochondrial antibodies, WBRKeyword::Pruritis, WBRKeyword::Fatigue
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