A 42-year-old man comes to the office due to progressive fatigue. For several years, the patient has had recurrent episodes of abdominal pain and diarrhea associated with bloody stools. Two years ago, he underwent a colonoscopy with biopsy, which demonstrated contiguous proctocolitis with pseudopolyps, mucosal ulcerations, and crypt abscesses. Oral 5-aminosalicylic acid and corticosteroid enemas controlled his symptoms, but he has had extreme fatigue for the past several months to where he feels drained by the end of the day. The patient has had no weight loss. Temperature is 37 C (98.6 F), blood pressure is 134/90 mm Hg, pulse is 74/min, and respirations are 15/min. Examination shows mild left lower quadrant tenderness on deep palpation. There is no hepatomegaly or splenomegaly. Laboratory results are as follows: Which of the following is the most appropriate next step in management?

Question

A 42-year-old man comes to the office due to progressive fatigue.  For several years, the patient has had recurrent episodes of abdominal pain and diarrhea associated with bloody stools.  Two years ago, he underwent a colonoscopy with biopsy, which demonstrated contiguous proctocolitis with pseudopolyps, mucosal ulcerations, and crypt abscesses.  Oral 5-aminosalicylic acid and corticosteroid enemas controlled his symptoms, but he has had extreme fatigue for the past several months to where he feels drained by the end of the day.  The patient has had no weight loss.  Temperature is 37 C (98.6 F), blood pressure is 134/90 mm Hg, pulse is 74/min, and respirations are 15/min.  Examination shows mild left lower quadrant tenderness on deep palpation.  There is no hepatomegaly or splenomegaly.  Laboratory results are as follows:  Which of the following is the most appropriate next step in management?

Quizplus · Accepted Answer

11ec25c8_d403_84ae_bc81_d5a000d68fcf_MD0003_00 This patient with fatigue and an elevated alkaline phosphatase most likely has primary sclerosing cholangitis (PSC). PSC is a chronic progressive disorder characterized by inflammation, fibrosis, and stricturing of intrahepatic and extrahepatic bile ducts. Affected individuals frequently have coexisting ulcerative colitis, explaining this patient's recurrent bloody diarrhea, anemia, left lower quadrant tenderness, and characteristic colonoscopy findings (eg, pseudopolyps, mucosal ulcerations, crypt abscesses). PSC is often asymptomatic but may present with fatigue and pruritus. Bile duct destruction results in cholestasis with characteristic laboratory abnormalities including markedly elevated alkaline phosphatase levels (and, eventually, bilirubin elevation) (ie, cholestatic pattern) with normal or minimally elevated serum aminotransferases. Other nonspecific findings may include elevated inflammatory markers (eg, erythrocyte sedimentation rate), increased IgM, and positive perinuclear antineutrophil cytoplasmic antibodies. The diagnosis is confirmed with imaging demonstrating multifocal bile duct strictures alternating with segments of ductal dilation (eg, beaded appearance). Magnetic resonance cholangiopancreatography (MRCP) is the imaging modality of choice due to its rapid, noninvasive nature; however, endoscopic retrograde cholangiopancreatography can be performed in patients who cannot undergo MRI (eg, those with metal implants). Although liver biopsy can be considered in patients with atypical presentations, the classic histologic findings associated with PSC (eg, ductal obliteration, periductular onion-skin fibrosis) are only present in 25% of liver biopsies (Choice E). multifocal bile duct strictures alternating with segments of ductal dilation (eg, beaded appearance) (Choice A) Anti-smooth muscle antibodies are among the autoantibody types seen in autoimmune hepatitis. This condition can cause nonspecific symptoms (eg, fatigue) but most commonly affects women and is associated with a hepatocellular pattern of liver injury (ie, elevated transaminases with mildly elevated or normal alkaline phosphatase). (Choice B) Biliary scintigraphy (HIDA scan) can diagnose acute cholecystitis; however, this presents with fevers, right upper quadrant pain, vomiting, and leukocytosis. Elevated liver function studies are not typical. (Choice C) Carcinoembryonic antigen levels are elevated in colon cancer, which can metastasize to the liver and cause biliary obstruction and cholestasis. Although ulcerative colitis and PSC increase the risk of developing colon cancer, patients with metastatic cancer typically have weight loss and hepatomegaly. (Choice D) CT scan is sometimes used to evaluate for malignancy (eg, cholangiocarcinoma), which can cause fatigue and, potentially, liver function abnormalities but is generally associated with weight loss and advanced age. Although CT scan of the abdomen can sometimes identify nonspecific bile duct dilation in patients with PSC, it is much less sensitive and specific when compared to MRCP. Educational objective: Primary sclerosing cholangitis is often asymptomatic but may present with fatigue and pruritus, and laboratory studies usually reveal cholestasis. It is strongly associated with ulcerative colitis. The diagnosis is confirmed with magnetic resonance cholangiopancreatography demonstrating multifocal bile duct strictures alternating with segments of ductal dilation (eg, beaded appearance). References: Primary sclerosing cholangitis: etiopathogenesis and clinical management. (http://www.ncbi.nlm.nih.gov/pubmed/22201985) Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease. (http://www.ncbi.nlm.nih.gov/pubmed/22407885)

A 42-Year-Old Man Comes to the Office Due to Progressive