AIDS cholangiopathy

Last revised by Andrea Molinari on 11 Mar 2022

AIDS cholangiopathy refers to an acalculous, secondary opportunistic cholangitis that occurs in AIDS patients as a result of immunosuppression 1

Pathology

Characterized by multiple irregular strictures essentially indistinguishable from primary sclerosing cholangitis (PSC). There are four pathological patterns 1,2

  • a combination of sclerosing cholangitis and papillary stenosis (50%)
  • isolated intrahepatic sclerosing cholangitis–like appearance (20%)
  • isolated papillary stenosis (15%)
  • long-segment extrahepatic duct stricture +/- concurrent intrahepatic disease (15%)
Etiology

No definite organism is identified in up to half of the patients. It typically affects patients with low CD4 counts (<135/mm3). Postulated causative organisms include 2:

  • cytomegalovirus (CMV) 
  • herpes simplex virus (HSV)
  • Cryptosporidium parvum
  • Microsporidium
  • Mycobacterium avium complex

Differential diagnosis

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