A 34-year-old man comes to the emergency department for right upper quadrant abdominal pain. The pain started abruptly 24 hours ago and is described as constant, severe, and progressive. The patient was hospitalized 6 months ago for similar symptoms that were resolved with antibiotics. Review of systems is positive for occasional bloody stools. There is no relevant family history, and the patient does not use tobacco, alcohol, or illicit drugs. Temperature is 39.8 C (103.6 F), blood pressure is 90/50, pulse is 120/min, and respirations are 22/min. The patient is lethargic and difficult to arouse. Scleral icterus is present. Cardiopulmonary examination is unremarkable. The abdomen is tender to palpation in the right upper quadrant. Laboratory evaluation reveals the following: Right upper quadrant ultrasound is normal. Which of the following is the most likely cause of this patient's presentation?

Question

A 34-year-old man comes to the emergency department for right upper quadrant abdominal pain.  The pain started abruptly 24 hours ago and is described as constant, severe, and progressive.  The patient was hospitalized 6 months ago for similar symptoms that were resolved with antibiotics.  Review of systems is positive for occasional bloody stools.  There is no relevant family history, and the patient does not use tobacco, alcohol, or illicit drugs.  Temperature is 39.8 C (103.6 F), blood pressure is 90/50, pulse is 120/min, and respirations are 22/min.  The patient is lethargic and difficult to arouse.  Scleral icterus is present.  Cardiopulmonary examination is unremarkable.  The abdomen is tender to palpation in the right upper quadrant.  Laboratory evaluation reveals the following:  Right upper quadrant ultrasound is normal.  Which of the following is the most likely cause of this patient's presentation?

Quizplus · Accepted Answer

11ec25c8_d882_a44d_bc81_2faca008b696_MD0003_00 This patient with right upper quadrant pain, fever, jaundice, hypotension, and altered mental status (ie, Reynolds pentad) has the typical features of acute cholangitis. Acute cholangitis is an infection of the extrahepatic biliary system that usually occurs due to biliary obstruction (eg, bile stone, malignancy, stricture), which predisposes patients to bacterial invasion of the normally sterile biliary tree. In a young adult male with a history of hematochezia, the most likely etiology of recurrent acute cholangitis (eg, similar symptoms in the past) is a biliary stricture due to primary sclerosing cholangitis (PSC). PSC is a chronic disease characterized by fibrosis and stricturing of the medium and large intra- and extrahepatic bile ducts. This promotes cholestasis (elevated alkaline phosphatase, hyperbilirubinemia) and obstruction. PSC usually affects men and is associated with ulcerative colitis, which explains this patient's hematochezia. Patients may have normal ultrasonography because intrahepatic bile ducts are not easily visible. Magnetic resonance cholangiopancreatography overcomes this limitation and is the preferred test to confirm the diagnosis in these patients. Management of PSC includes endoscopic interventions for strictures, therapy for coexisting ulcerative colitis, antibiotics for cholangitis (with some patients maintained on longterm antibiotic therapy), and, in some cases, ursodeoxycholic acid. (Choice A) Annular pancreas is a congenital disorder that results in a ringlike pancreas that encircles the duodenum. It can cause duodenal obstruction (eg, vomiting, pain, early satiety), but acute cholangitis is atypical. (Choice B) Cholangiocarcinoma is a risk factor for developing acute cholangitis and may develop as a complication of PSC; however, it usually develops in older patients (eg, age >50), and unexpected weight loss is typical. (Choice C) Chronic pancreatitis presents with recurrent abdominal pain, vomiting and steatorrhea. It typically occurs in patients with risk factors (eg, smoking, alcohol use, family history); it is not associated with acute cholangitis. (Choice D) Pancreatic divisum is a congenital disorder that results in the presence of a dorsal and ventral (rather than singular) pancreatic duct. Although it can cause recurrent acute pancreatitis (eg, abdominal pain, vomiting, elevated lipase), acute cholangitis is not expected. (Choice E) Primary biliary cholangitis (PBC) results from an immune response against the intrahepatic bile ducts can also present with cholestasis (eg, elevated alkaline phosphatase, jaundice, pruritus). However, it is much more common in women (~90% of cases) and is only rarely associated with ulcerative colitis. In addition, acute cholangitis (usually caused by obstruction of the extrahepatic bile ducts) is rare in PBC (which affects the intrahepatic bile ducts). Educational objective: Primary sclerosing cholangitis is characterized by fibrosis and stricturing of the medium and large intra- and extrahepatic bile ducts, promoting cholestasis and acute cholangitis. It occurs most commonly in men and is strongly associated with ulcerative colitis.

A 34-Year-Old Man Comes to the Emergency Department for Right