AIDS Cholangiopathy

Published on 19/07/2015 by admin

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Last modified 19/07/2015

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 Diffuse GB thickening ± sonographic Murphy sign in setting of acalculous cholecystitis

• MR: Papillary stenosis with tapered narrowing of distal CBD and proximal CBD dilatation

image ± long-segment extrahepatic bile duct strictures
image ± thickening and hyperenhancement of bile duct wall on T1WI C+ images in setting of acute inflammation
image Beaded appearance of intrahepatic ducts with alternating strictures, normal ducts, and dilated ducts
image GB wall thickening and pericholecystic inflammation
• ERCP: Papillary stenosis with proximal CBD dilation, strictures/ulcerations of CBD, and intrahepatic strictures

image Combination of ampullary stenosis and intrahepatic strictures is unique to AIDS cholangiopathy

TOP DIFFERENTIAL DIAGNOSES

• Primary sclerosing cholangitis
• Acute pyogenic cholangitis
• Secondary sclerosing cholangitis (IgG4-related, ischemic)
• Ampullary stenosis
• Cholangiocarcinoma

CLINICAL ISSUES

• Late-stage AIDS patients usually with CD4 < 100 cells/mm³
• Now considered rare condition due to HAART
• Patients present with RUQ pain, fever, weight loss, diarrhea, and markedly elevated alkaline phosphatase
• Primary treatment is reconstituting immune function using HAART, which can reverse cholangiopathy in some cases
image
(Left) Cholangiogram demonstrates a pruned appearance of the extrahepatic biliary tree with multiple beaded strictures, attributable in this case to AIDS cholangiopathy given the patient’s very low CD4 count.

image
(Right) Ultrasound of the portal hepatis in a man with Cryptosporidium cholangitis shows tapered narrowing of a dilated distal common bile duct (CBD) image and wall thickening image. (Courtesy K. Hosseinzadeh, MD.)
image
(Left) ERCP shows abnormal intrahepatic ductal arborization, with foci of stricture, dilation, and abrupt termination of ducts. The common duct image is dilated, and the distal duct is strictured image. Intrahepatic biliary strictures in AIDS cholangitis can resemble those seen in PSC.

image
(Right) MRCP shows strictured intrahepatic ducts image, extrahepatic biliary ductal dilatation, a narrowed distal CBD image, and papillary stenosis image. This combination is characteristic of AIDS cholangiopathy. (Courtesy V. Kabathina, MD.)

TERMINOLOGY

Synonyms

• AIDS- or HIV-related cholangitis, AIDS-related sclerosing cholangitis, AIDS-related cholangiopathy

Definitions

• Spectrum of biliary inflammation caused by AIDS-related opportunistic infections leading to biliary strictures/obstruction or acalculous cholecystitis

IMAGING

General Features

• Best diagnostic clue

image AIDS patient, typically with a very low CD4 count (< 100/mm³) with multiple intrahepatic strictures, ampullary stenosis, or gallbladder (GB) wall thickening from cholecystitis
• Location

image Can involve intrahepatic/extrahepatic ducts or GB
• Size

image Short or long segment biliary strictures
• Morphology

image Irregular intrahepatic strictures mimicking primary sclerosing cholangitis

Radiographic Findings

• ERCP: Papillary stenosis with proximal CBD dilation, CBD strictures/ulcerations, and multiple intrahepatic strictures

image Combination of ampullary stenosis and intrahepatic strictures is unique to AIDS cholangiopathy
• ERCP is gold-standard for diagnosis but carries risks related to invasive procedure

CT Findings

• CECT

image Dilatation of CBD with tapered narrowing of distal CBD 

– ± thickening and hyperenhancement of CBD wall
image Beaded appearance of intrahepatic ducts: Alternating sites of narrowing, normal ducts, and dilated ducts
image Gallbladder (GB) wall thickening and pericholecystic fat stranding in patients with acalculous cholecystitis

MR Findings

• MR/MRCP very sensitive (85-100%) and specific (92-100%) 

image Allows visualization of proximal ducts even in setting of tight strictures (advantage over ERCP)
• Papillary stenosis with tapered narrowing of distal CBD (without abrupt margins) and proximal CBD dilatation

image ± long-segment extrahepatic bile duct strictures

– ± thickening and hyperenhancement of bile duct wall on T1WI C+ in setting of acute inflammation
image Pancreatic duct usually normal in caliber
• Intrahepatic ductal strictures resembling PSC

image Multifocal sites of alternating strictures, normal caliber ducts, and dilated ducts producing beaded appearance
image Greater than expected visualization of intrahepatic ducts on MRCP should suggest intrahepatic strictures
• GB wall thickening and pericholecystic inflammatory changes in patients with acalculous cholecystitis

Ultrasonographic Findings

• Dilated intrahepatic ducts with CBD thickening and periductal hyper-/hypoechoic areas 

image Usually smooth tapered narrowing of distal CBD in setting of papillary stenosis
• Diffuse GB thickening ± sonographic Murphy sign in the setting of acalculous cholecystitis

Imaging Recommendations

• Best imaging tool

image US: Screening modality for biliary ductal dilatation
image MRCP: Best noninvasive modality
image ERCP: Gold standard for establishing diagnosis and excluding malignancy at strictures using cytologic brushing
• Protocol advice

image US protocol: Parasagittal view of CBD to demonstrate mural thickening
image MRCP protocol: Axial and coronal single-shot fast spin-echo (SSFSE); obliques, heavily T2WI

DIFFERENTIAL DIAGNOSIS

Primary Sclerosing Cholangitis

• Chronic inflammatory disorder involving the intrahepatic and/or extrahepatic bile ducts
• 

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