A 32-year-old man comes to the office due to malaise and fatigue over the last 3 months. The patient also reports mild generalized abdominal discomfort and nausea. He has lost approximately 2.3 kg (5 lb) in the last month. The patient has had no fever, vomiting, diarrhea, back pain, or urinary symptoms. He has no medical issues and takes no medication. The patient is a lifetime nonsmoker. Family history is notable for liver cancer in his father.
Physical examination shows right upper quadrant tenderness. The liver edge is soft and palpable 2 cm below the right costal margin. Rectal examination shows no abnormalities, and a stool occult blood test is negative.
Laboratory results are as follows:
A hepatitis panel shows immunity against the hepatitis B virus. Ultrasonography shows no gallstones and no common bile duct dilation. Liver biopsy reveals intrahepatic ductal obliteration with moderate lymphocytic infiltration and periductal "onion skin" fibrosis.
Which of the following is the best next step in management of this patient?

Question

A 32-year-old man comes to the office due to malaise and fatigue over the last 3 months.  The patient also reports mild generalized abdominal discomfort and nausea.  He has lost approximately 2.3 kg (5 lb) in the last month.  The patient has had no fever, vomiting, diarrhea, back pain, or urinary symptoms.  He has no medical issues and takes no medication.  The patient is a lifetime nonsmoker.  Family history is notable for liver cancer in his father.
Physical examination shows right upper quadrant tenderness.  The liver edge is soft and palpable 2 cm below the right costal margin.  Rectal examination shows no abnormalities, and a stool occult blood test is negative.
Laboratory results are as follows:
 A hepatitis panel shows immunity against the hepatitis B virus.  Ultrasonography shows no gallstones and no common bile duct dilation.  Liver biopsy reveals intrahepatic ductal obliteration with moderate lymphocytic infiltration and periductal "onion skin" fibrosis.
Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

11ec193a_97f4_4c3e_853d_134198ef2921_MD0002_00 This patient's presentation is consistent with cholestasis due to primary sclerosing cholangitis (PSC), which is associated with underlying inflammatory bowel disease (IBD) (usually ulcerative colitis) in nearly 90% of patients. PSC is a chronic progressive disorder of unknown etiology with inflammation, fibrosis, and stricturing, most commonly of medium and large biliary ducts. Patients are usually asymptomatic but can present with fatigue, pruritus, and a cholestatic pattern of elevations in liver function tests. Continued bile duct destruction leads to end-stage liver disease and portal hypertension. Ultrasound is usually nondiagnostic; a cholangiogram (either endoscopic or magnetic resonance) may show multifocal narrowing and dilation of both intrahepatic and extrahepatic bile ducts. Liver biopsy is helpful for disease staging and prognosis, and can reveal intrahepatic ductal obliteration with moderate lymphocytic infiltration and periductal "onion skin" fibrosis. PSC patients with IBD are at significantly increased risk of colorectal cancer. Current guidelines recommend colonoscopy with full biopsies in newly diagnosed patients with PSC regardless of the presence of other symptoms. Patients found to have IBD require yearly follow-up with colonoscopy; those with PSC but without IBD should have colonoscopy every 5 years. Liver transplantation is the preferred treatment for PSC as medical therapy (eg, cyclosporine) does not appear to slow progression of the disease (Choice B). (Choice C) Although glucocorticoids are an important treatment for IgG4-related cholangitis, they have not been shown to improve survival in PSC and can worsen bone loss. (Choice D) Patients with PSC are also at increased risk for cholangiocarcinoma; some authorities have advocated for annual monitoring of cancer antigen 19-9 levels for screening. However, the evidence in support of this practice is not conclusive. (Choice E) Ursodeoxycholic acid is a mainstay of treatment for primary biliary cholangitis (PBC), which can present similarly to PSC. However, PBC usually affects women (90% of cases), and biopsy shows mononuclear cell-mediated destruction and/or periductal granulomatous inflammation of intrahepatic bile ducts. Ursodeoxycholic acid does not have a clear role in management of PSC. Education objective: Primary sclerosing cholangitis presents with cholestasis and can reveal intrahepatic ductal obliteration with moderate lymphocytic infiltration and periductal "onion skin" fibrosis. Most patients have comorbid inflammatory bowel disease (IBD) (usually ulcerative colitis) and a significantly increased risk of colorectal cancer. Colonoscopy is recommended in all patients to diagnose underlying IBD and for colorectal cancer surveillance. __________ References: Primary sclerosing cholangitis. (http://www.ncbi.nlm.nih.gov/pubmed/27653566)

A 32-Year-Old Man Comes to the Office Due to Malaise