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+A 68-year-old female nonsmoker, nondrinker with a medical history of hypertension presented with new-onset painless jaundice and pruritus, a three-month history of 9.9 kg weight loss and chronic diarrhea with four to five loose bowel movements per day.
+Medications included vitamin D, amlodipine and eprosartan.
+Physical examination was normal except for jaundice and muscle wasting.
+Recent colonoscopy had been normal.
+Total and direct bilirubin levels were 6.84 mg/dL (116.96 μmol/L) and 9.18 mg/dL (156.98 μmol/L), respectively.
+Other results included an international normalized ratio of 1.0, alanine aminotransferase level 247 U/L (normal <33 U/L), aspartate aminotransferase level 139 U/L (normal <32 U/L) and alkaline phosphatase level 524 U/L (normal 35 to 104 U/L).
+Viral hepatitis serologies, and antimitochondrial antibody and anti-smooth muscle antibody tests were negative.
+Her alpha-fetoprotein level was 2.4 ng/mL (normal <5 ng/mL), total immunoglobulin (Ig) G was 1880 mg/dL (normal <640 mg/dL), carbohydrate antigen 19-9 was 856 U/mL (normal <33 U/mL) and IgG4 was 890 g/L (normal <3 g/L).
+Doppler ultrasound, magnetic resonance cholangiopancreatography and magnetic resonance imaging of the liver were suspicious for a subtly enhancing mass (2.8 cm to 4.2 cm in diameter) in the region of the hilum and porta hepatis, obstructing both the right and left hepatic ducts.
+Endoscopic retrograde cholangiopancreatography identified strictures in the central portions of the right and left hepatic duct, which was concerning for cholangiocarcinoma (Figure 1).
+Biliary brushings were negative for malignancy.
+Esophagogastroduodenoscopy was normal.
+Biopsies of the ampulla of Vater revealed chronic active duodenitis (Figures 2 and ​and3);3); an ancillary test confirmed the diagnosis (Figure 4).
+Treatment with corticosteroids normalized the patient’s biochemical and radiological abnormalities within three months.