University of South Alabama University Hospital Mobile, AL
Yasir Ahmed, MD1, Mustafeez Ur Rahman2, Eduardo Calderon, MD1; 1University of South Alabama University Hospital, Mobile, AL; 2USA UH, Mobile, AL
Introduction: Cholangiopathy in Acquired Immune Deficiency Syndrome (AIDS) is less frequently reported since antiretroviral therapy (ART) is more readily available. It is seen with advanced disease and in patients with a history/presence of opportunistic infection.
Methods: Case 1:28-years-old female with history of intravenous drug use and AIDS presented with right upper quadrant (RUQ) pain and increasing generalized abdominal swelling. Laboratory work-up showed cholestatic picture and a CD4 count of 18 (463-1685/mcL), abdominal ultrasound (U/S) revealed liver cirrhosis and a normal biliary structure and polymerase chain reaction (PCR) was positive for cytomegalovirus (CMV). Liver biopsy showed portal inflammation, cholangitis and ductular reaction confirming AIDS cholangiopathy. Case 2:38-year-old male with AIDS was transferred from group home with failure to thrive. He appeared severely cachectic, and was tender in RUQ and epigastric region. Laboratory studies showed cholestatic picture with elevated bilirubin of 9.0 mg/dL (normal 0.2-1.0 mg/dL), a CD4 count of 241 while viral load was 791 copies/mL. Stool was positive for cryptosporidium and PCR positive for CMV. There was no evidence of biliary ductal dilation on U/S or magnetic resonance cholangiopancreatography (MRCP). Liver biopsy showed cholestatic hepatitis with marked inflammation, prominent ductular proliferation and portal septal fibrosis with focal bridging suggesting AIDS cholangiopathy. He passed away within a month.Case 3:27 years old male with AIDS presented with RUQ abdominal pain and diarrhea. Laboratory work-up showed thrombocytopenia, cholestatic picture and a new diagnosis of AIDS with aCD4 count of 38. CMV was detected by PCR in blood and also seen in rectal tissue biopsy, confirming colitis in the setting of disseminated CMV. Although U/S and MRCP were normal, endoscopic retrograde cholangiopancreatography (ERCP) showed edematous and strictured common bile duct from the distal to proximal portion and its bifurcation and right and left intrahepatic ducts were missing.
Discussion: The most common opportunistic organisms associated with AIDS cholangiopathy are Cryptosporidium parvum and CMV. Cholangiography is the test of choice for diagnosis. The yield of liver biopsy is low. Its recognition becomes important as the prognosis of AIDS cholangiopathy is generally not favorable, therapy for opportunistic infections is mostly ineffective and restoration of immune system with ART remains the therapy of choice.
Disclosures: Yasir Ahmed indicated no relevant financial relationships. Mustafeez Ur Rahman indicated no relevant financial relationships. Eduardo Calderon indicated no relevant financial relationships.