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, 13 (1), 153-158
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Sarcoidosis of the Intra- And Extrahepatic Bile Ducts With Concomitant Cholangitis in a Patient With Ulcerative Colitis

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Case Reports

Sarcoidosis of the Intra- And Extrahepatic Bile Ducts With Concomitant Cholangitis in a Patient With Ulcerative Colitis

Ahmad Fasel Jebran et al. Case Rep Gastroenterol.

Abstract

Cholangitis in patients with ulcerative colitis (UC) can lead to misdiagnosis of primary sclerosing cholangitis (PSC). Furthermore, it can mimic cholangiocellular carcinoma, which also can lead to inappropriate and potentially harmful treatment of the patient. An 18-year-old male patient with known UC presented with pain in his right upper abdomen and elevation of the cholestatic liver enzymes (alkaline phosphatase: 197 U/L, γ-glutamyltransferase: 229 U/L) and increased inflammatory parameters (leukocytosis and CrP of 13.6 mg/L). Magnetic resonance cholangiopancreatography revealed unclear stenosis in the bifurcation of the main hepatic bile duct as well as in the prepapillary bile duct. Ultrasound (US) examination and endoscopic retrograde cholangiopancreatography showed dilatation of the intra -and extrahepatic bile ducts, which raised the suspicion of PSC. US image with dilated intra- and extrahepatic dilatation of the bile duct was also suggestive for autoimmune cholangitis. However, serum analysis revealed an elevated soluble interleukin-II receptor (1,305 U/mL), while immunoglobulin G4 was within normal ranges. Liver biopsy demonstrated hepatic inflammation and presence of granulomatous cells within the portal fields - convenient to sarcoidosis. After starting treatment with steroids, we observed a rapid clinical response with improvement of the dilated bile ducts and decrease of the initially elevated cholestatic liver enzymes. Sarcoidosis within the bile duct is a rare condition. Steroids are the treatment of choice and - along with the histology - are furthermore helpful to differentiate between several potential differential diagnoses like IgG4 cholangitis, primary biliary cholangitis, or PSC.

Keywords: IgG4 cholangitis; Inflammatory bowel disease; Primary sclerosing cholangitis; Sarcoidosis; Ulcerative colitis.

Figures

Fig. 1
Fig. 1
Endoscopic retrograde cholangiopancreatography demonstrating constriction of the main duct bifurcation and the prepapillary region (marked with arrows).
Fig. 2
Fig. 2
Diameter of the main bile duct before prednisolone therapy initially measuring 12 mm (left panel) and following 4 days of prednisolone therapy with significant reduction to 6 mm (right panel).
Fig. 3
Fig. 3
Magnification of 200–400×, section 1 stained with HE, section 2 after immunohistochemistry against CK7 showing destruction of the bile ducts and the formation of epithelioid cells around the bile ducts.

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