Vanishing bile duct syndrome in a patient with advanced AIDS

Abstract

A 39-year-old HIV-infected woman developed signs and symptoms of obstructive jaundice and cholestasis. Serological tests were positive for cytomegalovirus (CMV) infection. There was no evidence of AIDS cholangiopathy in ultrasonography or magnetic resonance cholangiopancreatography (MRCP). A liver biopsy revealed marked ductopenia and the patient was diagnosed with vanishing bile duct syndrome, thought to be secondary to CMV infection as a result of profound immunosuppression. To the best of our knowledge, this is the first reported case of vanishing bile duct syndrome diagnosed in a patient with HIV/AIDS.Keywords: ductopenia, idiopathic adulthood ductopenia, intrahepatic cholestasis, vanishing bile duct syndrome Case reportA 39-year-old woman presented with a complaint of fatigue, anorexia, nausea and vomiting, of duration 3 days. She also reported a 1-week history of scleral icterus, pruritis and abdominal pain. The nausea and vomiting were described as nonbilious and nonbloody, occurring three times a day, while the abdominal pain was localized to the right upper quadrant without radiation or association with meals. Past medical history was significant for HIV infection and hepatitis C virus (HCV) infection diagnosed in 1997. She was not taking any medication, including highly active antiretroviral therapy (HAART) or treatment for HCV infection, because of a history of polysubstance abuse of cocaine, alcohol and intravenous drugs. Her last documented CD4 cell count and quantitative HIV-1 RNA viral load 7 months prior to admission were 22 cells/mL (3%) and 48 026 HIV RNA copies/mL, respectively. Her weight was 43 kg and all initial vital signs were normal.Physical examination was significant for cachexia and generalized icterus. The right upper quadrant was tender to palpation without rebound tenderness or guarding. There were no masses palpated and no evidence of ascites. Murphy's sign was negative. The liver span was measured as 5 cm. No cutaneous manifestations of chronic liver disease were noted. Initial chemistry values were significant for the following: albumin 5 2.9 g/dL, total bilirubin 5 15.4 mg/dL, direct bilirubin 5 11.0 mg/dL, alkaline phosphatase 5 2200 IU/L, aspartate transaminase 5 114 IU/ L and alanine transaminase 5 29 IU/L. The partial thromboplastin time and international normalized ratio were 39 s and 1.4, respectively. Urine analysis revealed proteinuria and bilirubinuria while the CD4 cell count and HIV-1 RNA viral load were measured as 7 cells/mL (2%) and 721 000 copies/mL, respectively. HCV RNA measured by quantitative PCR was 45 000 000 IU/mL, while the a-fetoprotein level was o5 ng/mL. Other haematological values were not significant and ethanol and urine drug screens were negative.The patient's abdominal complaints did not change during the course of her admission and an abdominal ultrasound revealed no evidence of cirrhosis, focal liver lesions, intrahepatic duct dilatation or hepatic vein thrombosis. The common bile duct was measured and found to be normal. Fungal, mycobacterium,...

Liver Disease in the HIV–Infected Individual

“…Multiple causes have been identified, and there have been case reports of VBDS associated with advanced AIDS, with cases attributed to CMV viremia and medication toxicity [98, 99] . The presentation is variable and often related to cholestasis.…”

Immunobiology of Human Cytomegalovirus: from Bench to Bedside

“…In addition to directly causing end-organ disease, HCMV has also been associated with a number of damaging indirect effects in SOT patients. HCMV has been implicated in increased graft rejection (70,94,223) and is associated with renal artery stenosis in renal transplant recipients (11,203), accelerated coronary artery stenosis in heart transplant recipients (137,170), bronchiolitis obliterans in lung transplant recipients (13,140), and vanishing bile duct syndrome in liver transplant recipients (7,106,146,187); however, a causative role of the virus remains to be established. Furthermore, HCMV infection can also predispose transplant patients to opportunistic superinfection with a range of different microorganisms including Pneumocystis carinii, a variety of fungi, and Listeria monocytogenes (82,241).…”

Ischemic Type Biliary Lesions

“…Finally, graft atrophy and the reduction of functional reserve may develop, compromising the result of an initially successful LT. Significant morphological similarities are observed in a non-transplant setting as well: in patients with HIV-associated cholangiopathy as vanishing bile duct-syndrome, sclerosing cholangitis and shock-liver [1,26]. In summary, chronic inflammation caused by any noxious effectors may lead to the uniform picture described above.…”