A 46-year-old woman was instructed to follow up with her primary care physician after a recent emergency department visit for self-limited nausea and vomiting. The patient currently has no issues. Her medical history is notable for obesity, ulcerative colitis, essential hypertension, and gastroesophageal reflux disease. The patient's last ulcerative colitis flare was 5 years ago and was treated with a short course of corticosteroids. Colonoscopy with biopsy performed a year ago revealed mild inflammation with no dysplasia. Her medications include mesalamine, omeprazole, and lisinopril.
Blood pressure is 140/88 mm Hg, pulse is 84/min, and respirations are 14/min. BMI is 32 kg/m². Cardiopulmonary examination is normal. The abdomen is soft and nontender. There is no hepatomegaly or splenomegaly.
Laboratory results from the patient's recent emergency department visit are as follows:
Repeat testing shows similar results.
Which of the following would be the most helpful for establishing the diagnosis in this patient?

Question

A 46-year-old woman was instructed to follow up with her primary care physician after a recent emergency department visit for self-limited nausea and vomiting. The patient currently has no issues. Her medical history is notable for obesity, ulcerative colitis, essential hypertension, and gastroesophageal reflux disease. The patient's last ulcerative colitis flare was 5 years ago and was treated with a short course of corticosteroids. Colonoscopy with biopsy performed a year ago revealed mild inflammation with no dysplasia. Her medications include mesalamine, omeprazole, and lisinopril.
Blood pressure is 140/88 mm Hg, pulse is 84/min, and respirations are 14/min. BMI is 32 kg/m². Cardiopulmonary examination is normal. The abdomen is soft and nontender. There is no hepatomegaly or splenomegaly.
Laboratory results from the patient's recent emergency department visit are as follows:

Repeat testing shows similar results.
Which of the following would be the most helpful for establishing the diagnosis in this patient?

Quizplus · Accepted Answer

11ec193a_9806_e911_853d_f174e8ca3241_MD0002_00 This patient with ulcerative colitis has a cholestatic injury pattern on liver function tests (elevated alkaline phosphatase > transaminases). This is highly suggestive of primary sclerosing cholangitis (PSC). More than 90% of patients with PSC have underlying ulcerative colitis. PSC involves chronic inflammation and fibrosis of the hepatic duct system, leading to strictures and eventually cirrhosis. The majority of patients are diagnosed due to asymptomatic alkaline phosphatase elevation; however, progressive disease can cause abdominal pain, fatigue, pruritus, weight loss, and mild aminotransferase elevation (<300 U/L). In rare instances, patients may have overt cholangitis. Diagnosis is made with cholangiography demonstrating multifocal stricturing and focal dilation of hepatic bile ducts: MR cholangiopancreatography (MRCP) is often the imaging of choice due to its noninvasive natureEndoscopic retrograde cholangiopancreatography (ERCP) can be used to evaluate very early disease or in patients who cannot tolerate MRI (eg, metal implants, pacemaker) MR cholangiopancreatography (MRCP) is often the imaging of choice due to its noninvasive nature MR cholangiopancreatography (MRCP) is often the imaging of choice due to its noninvasive nature (MRCP) Endoscopic retrograde cholangiopancreatography (ERCP) can be used to evaluate very early disease or in patients who cannot tolerate MRI (eg, metal implants, pacemaker) Endoscopic retrograde cholangiopancreatography (ERCP) can be used to evaluate very early disease or in patients who cannot tolerate MRI (eg, metal implants, pacemaker) (ERCP) Liver biopsies are rarely diagnostic; the specific histologic finding of an "onion skin" pattern of fibrous obliteration is seen in a minority of patients. In most cases (>98% in one study), biopsy results do not change management; biopsies are typically reserved for patients with high suspicion for small duct PSC (ie, characteristic histologic findings with normal cholangiographic findings) or an overlap syndrome (Choice D). (Choice A) Alkaline phosphatase is also elevated in diseases with rapid bony turnover (eg, Paget, osteomalacia), and bone scan can be helpful for diagnosis. However, the high gamma glutamyltransferase level indicates a hepatic source of the alkaline phosphatase. (Choice C) CT scans can be used to evaluate for malignancy or a mass causing cholestasis; however, they are relatively nonspecific in differentiating between malignancy and PSC. Compared to CT, MRCP is more sensitive and specific in diagnosis of cholestatic liver disease. In addition, ultrasound is typically preferred initially over CT scan for evaluating cholestatic liver injury as it is inexpensive, lacks radiation exposure, and can accurately measure biliary duct dilation. (Choice E) Mesalamine can very rarely cause cholestatic liver injury. However, patients with ulcerative colitis are at increased risk of developing PSC; if cholestatic liver injury is noted, PSC must be ruled out. Educational objective: Ulcerative colitis is strongly associated with primary sclerosing cholangitis, which should be suspected in those with cholestatic liver injury. Diagnosis is made by findings of multifocal stricturing and dilation of hepatic bile ducts on MR or endoscopic retrograde cholangiopancreatography. __________ References: Primary sclerosing cholangitis. (http://www.ncbi.nlm.nih.gov/pubmed/20955968)

A 46-Year-Old Woman Was Instructed to Follow Up with Her