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lymphocytes, and an outer layer of fibrosis. This disease may respond to erythromycin
therapy.[508,510,889,890]
Veno-occlusive disease (VOD) has been reported in patients with AIDS, particularly in
persons with a risk factor for HIV infection of injection drug use. Pathologic findings of VOD
are central vein obliteration and sclerosis, sinusoidal congestion and fibrosis, and perivenular
hepatocellular degeneration and necrosis. The occurrence of VOD with AIDS may be related to
the effects of multiple drugs.[891]
BILIARY TRACT.-- The biliary tract and gallbladder with AIDS may occasionally be
involved by a variety of lesions including acalculous cholecystitis, sclerosing cholangitis, and
papillary stenosis. Collectively, these lesions are known as AIDS cholangiopathy, and most
patients who exhibit these findings have a CD4 count <200/µL. Acalculous cholecystitis is
suggested by right upper quadrant or epigastric pain and low-grade fever, though jaundice is not
common. Liver function tests demonstrate markedly elevated alkaline phosphatase, moderate
transaminase elevation, and a normal or slightly increased bilirubin. Acalculous cholecystitis is
accompanied by marked dilation and edema with thickening of the gallbladder wall, bile duct
dilation, intrahepatic duct dilation, and cholestasis seen on ultrasonography. Ultrasonography
can identify nearly all cases of AIDS cholangiogpathy and select cases for endoscopic retrograde
pancreatography. Findings may include a dilated common bile duct and narrowing in the distal
duct consistent with papillitis or papillary stenosis. Intrahepatic ductal strictures are sometimes
seen. Edema, necrosis, and ulceration can be seen pathologically. Endoscopic sphincterotomy
provides relief of abdominal pain and resolution of obstruction. Cholangiography may regress
with antiretroviral therapy.[871,873,892,893]
Infectious agents including Cryptosporidium, Enterocytozoon bieneusi and Septata
intestinalis, and cytomegalovirus have been identified in patients with AIDS cholangiopathy or
cholecystitis. About one fourth of AIDS patients undergoing cholecystectomy have gallstones.
Nodules of Kaposi's sarcoma may occur, usually with widespread disease, at the liver hilum and
lead to biliary tract obstruction, as can enlarged lymph nodes from MAC infection.[894]
AIDS cholangiopathy may manifest as a secondary sclerosing cholangitis that is
suggested by the appearance of epigastric pain, fever, diarrhea, and increased alkaline
phosphatase. The bilirubin is usually not elevated. Diagnosis can be made by endoscopic
retrograde pancreatography (ERCP), which may demonstrate stricturing, dilation, and beading of
the biliary tract. Ultrasonography is often abnormal. At the time of ERCP, abnormalities of the
pancreatic duct may also be apparent in half of cases. Sphincterotomy of the papilla of Vater
may provide symptomatic relief. Liver biopsy can also be helpful for diagnosis. This disease
appears to have no influence upon the prognosis with AIDS.[873,895]