Article Text

Rare disease
Obstructive jaundice due to autoimmune cholangiopathy
  1. Osama Naji1,
  2. Abdulzahra Hussain2,
  3. Dennis Baker3,
  4. Naji Habib4,
  5. Shamsi El-Hasani1
  1. 1
    Princess Royal University Hospital, General Surgery Department, Farnborough Common, Orpington BR6 8ND, UK
  2. 2
    Princess Royal University Hospital, Farnborough Common, Orpington BR6 8ND, UK
  3. 3
    Princess Royal University Hospital, Radiology Department, Farnborough Common, Orpington BR6 8ND, UK
  4. 4
    Hepatobiliary Unit, Hammersmith Hospital, London W12 0NN, UK
  1. Abdulzahra Hussain, azahrahussain{at}


A 59-year-old man presented with upper abdominal pain, cholestasis and radiological evidence of common hepatic duct hilar stricture which was suggestive of cholangiocarcinoma. The patient initially underwent percutaneous drainage and a laparotomy. No evidence of malignancy was identified. He was noted to have retroperitoneal fibrosis, which was confirmed on histology. The combination of cholangiopathy and retroperitoneal fibrosis suggested an underlying autoimmune process. Although the investigations did not show any evidence of IgG4 related disease, the combination of a cholangiopathy and retroperitoneal fibrosis is in keeping with autoimmune cholangiopathy and a steroid regimen was commenced. Our patient is now symptom-free with no further episodes of cholangitis. He has commenced azathioprine to maintain long term remission.

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  • Competing interests: none.

  • Patient consent: Patient/guardian consent was obtained for publication

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