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AIDS Cholangiopathy

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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
• Beaded and pruned appearance of intrahepatic ducts with asymmetric short segment strictures alternating with normal sized ducts and sites of saccular dilatation

image Involvement of intrahepatic ducts is indistinguishable from AIDS cholangiopathy without clinical history
• Can also involve CBD with beaded strictures and pseudodiverticula, but no papillary stenosis
• Involvement of cystic duct characteristic

Acute Pyogenic Cholangitis

• Usually results from distal CBD obstruction
• Biliary dilatation with hyperenhancement/thickening of bile duct walls and heterogeneous liver enhancement

IgG4-Related Sclerosing Cholangitis

• Infiltration of biliary tree by IgG4-positive plasma cells
• Can result in strictures of intrahepatic or extrahepatic ducts
• Strictures tend to be long and continuous with greater propensity for proximal obstruction/dilatation

Ischemic Cholangitis

• Usually after liver transplant or surgical injury to vasculature
• Multiple intrahepatic and extrahepatic strictures may appear identical to PSC or AIDS cholangiopathy

Ampullary Stenosis

• Smooth distal CBD stricture without ulceration possibly due to passage of CBD stones or chronic pancreatitis

Acalculous Cholecystitis

• Gallbladder wall thickening with pericholecystic fluid, inflammation, and positive sonographic Murphy sign (but no gallstones)

image Not associated with AIDS or opportunistic infection
• Often secondary to ischemic injury from low-flow state

image Post-cardiac surgery
• Percutaneous cholecystomy for patients with poor operative risk
• Late in clinical course

image Secondary to bacterial invasion

Cholangiocarcinoma

• Short segment stricture with focal soft tissue thickening or discrete mass and progressive proximal biliary obstruction

image Associated with primary sclerosing cholangitis, recurrent pyogenic cholangitis (RPC), and choledochal cyst
• Infiltrates along ductal epithelium, invades hepatic parenchyma
• Delayed CECT useful to demonstrate intrahepatic component

PATHOLOGY

General Features

• Etiology

image AIDS-related opportunistic infection of biliary tract

– Cryptosporidium, Microsporidia, cytomegalovirus (CMV), and  Cyclospora most common
– No pathogen identified in 50% of cases
image CMV has been implicated in vasculitis of CNS, retinal, or gastrointestinal (GI) tract
image Strictures may also be due to CMV-related vasculitis
• Genetics

image No known predisposition
• Associated abnormalities

image Opportunistic infection of GB and bile ducts from Cryptosporidium and CMV, periductal inflammation, acalculous cholecystitis
image Other AIDS-related GI infections

– Enteritis and colitis from CMV, Cryptosporidium, and other opportunistic infections
– Mycobacterium avium intracellulare can cause ileocecal inflammation and necrotic mesenteric nodes
– Hepatic and splenic microabscesses from fungal infections (Candida, Cryptococcus)
image AIDS-related malignancies

– Non-Hodgkin lymphoma of liver, stomach, spleen, mesentery, or retroperitoneal nodes
– Kaposi sarcoma of retroperitoneal nodes

Staging, Grading, & Classification

• Type I (15-20%): Distal CBD stricture from papillary stenosis
• Type II (20%): Diffuse intrahepatic biliary strictures
• Type III (50%): Combined types I and II
• Type IV (15%): Long segment stricture of CBD ± intrahepatic ductal involvement
• Type V: Acalculous cholecystitis

Gross Pathologic & Surgical Features

• Acalculous cholecystitis, biliary strictures involving CBD, intrahepatic duct
• Biliary strictures involving CBD, intrahepatic duct

Microscopic Features

• CMV inclusions, Cryptosporidium organisms may be found on biopsy
• Fibrotic strictures of CBD, intrahepatic ducts

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image RUQ pain (most common with papillary stenosis)
image Fever (∼ 33%) and jaundice (10-20%)

– Jaundice uncommon, since biliary obstruction usually incomplete
image Weight loss
• Other signs/symptoms

image Diarrhea: Due to parasitic involvement of small bowel
• Clinical profile

image Significantly ↑ alkaline phosphatase
image Mildly ↑ bilirubin, ↑ GGT, and ↑ AST/ALT

– Abnormal liver function test can be due to other concomitant conditions (e.g., viral or drug-induced hepatitis)
image Cryptosporidium in stool or duodenal aspirate

Demographics

• Age

image Median 40-50 years
• Gender

image M < F
• Ethnicity

image Parallel demographics for AIDS patients
• Epidemiology

image Late-stage AIDS (classically CD4 < 100 cells/mm³)

– 20% of cases with higher CD4 counts, possibly on basis of resistance to first-line antiretroviral drugs
image Incidence has decreased substantially with highly active antiretroviral therapy (HAART)
image Prevalence was 24% before introduction of HAART
image Now considered a rare condition in AIDS population due to highly active antiretroviral therapy (HAART)

Natural History & Prognosis

• Primary treatment is reconstitution of immune function using HAART, which can reverse cholangiopathy (including imaging findings) in some cases

image Antimicrobial therapy usually ineffective
• Sphincterotomy provides some pain relief but does not alter intrahepatic disease or mortality
• Poor prognosis since AIDS cholangiopathy is usually seen in patients with advanced AIDS

image Mortality is due to natural history of AIDS

Treatment

• Options, risks, complications
• Primary treatment is reconstitution of immune function using HAART, which can reverse cholangiopathy (including imaging findings) in some cases

image Antimicrobial therapy usually ineffective 

– Does not affect symptoms or cholangiographic abnormalities
image Controversial role for ursodeoxycholic acid (Ursodiol)
• Sphincterotomy in symptomatic patients with papillary stenosis ± stent placement for dominant strictures

DIAGNOSTIC CHECKLIST

Consider

• AIDS cholangiopathy may appear very similar to other forms of sclerosing cholangitis (primary sclerosing cholangitis, ischemic cholangitis, IgG4 cholangitis)

Image Interpretation Pearls

• AIDS patient with distal ampullary stenosis, intrahepatic strictures, or acalculous cholecystitis
image
Sagittal ultrasound of the common bile duct demonstrates diffuse thickening representing edema from AIDS cholangitis.

image
Axial ultrasound of the common bile duct shows irregular thickening image from AIDS cholangitis.
image
Sagittal ultrasound demonstrates diffuse gallbladder wall thickening image in CMV acalculous cholecystitis.
image
Axial CECT demonstrates gallbladder wall edema and pericholecystic inflammatory changes image in CMV acalculous cholecystitis.
image
Sagittal ultrasound of the distal common bile duct demonstrates focal mural thickening image in ampullary stenosis from Cryptosporidium in AIDS cholangitis.
image
ERCP of a patient with ampullary stenosis from Cryptosporidium in AIDS cholangitis demonstrates a distal common bile duct stricture image.
image
Ultrasound image demonstrates a focal stricture of the common bile duct, as well as focal wall thickening of the duct further downstream image, typical features of AIDS-related cholangiopathy.
image
Coronal MRCP MIP image demonstrates multiple irregular strictures in the intrahepatic ducts, as well as low signal stones image within the intrahepatic ducts. The appearance of the intrahepatic ducts in this case can be seen with any form of sclerosing cholangitis, in this case due to AIDS cholangiopathy. Hepatolithiasis in this case is an unusual feature.
image
Cholangiogram image demonstrates multiple strictures of the intrahepatic biliary tree, which appear “beaded” and irregular, as well as hepatolithiasis image within a focally dilated bile duct segment.
image
Sagittal ultrasound demonstrates peripheral biliary ductal dilatation image as well as intrahepatic ductal stones image in a patient with AIDS-related cholangiopathy.
image
Ultrasound of the porta hepatis in a young HIV-positive man with confirmed Cryptosporidium cholangitis shows a dilated common duct and irregular wall thickening image. (Courtesy K. Hosseinzadeh, MD.)
image
ERCP of AIDS cholangitis in a 44-year-old man demonstrates multiple intrahepatic biliary strictures and irregular ductal contours image. Stool cultures were positive for Cryptosporidium.
image
Coronal oblique MRCP of AIDS-related cholangiography shows findings similar to those seen with ascending or sclerosing cholangitis. Although characteristic of AIDS-related cholangiopathy, it is important to know the clinical setting. Note the dilated common bile duct with distal CBD papillary stenosis image.
image
Another coronal MRCP shows dilation of the intrahepatic and extrahepatic bile ducts. Note the irregular branching pattern of the intrahepatic ducts image, signifying cholangitis. The dilation of the common bile duct (CBD) image is probably due to a distal CBD stricture.
image
Frontal ERCP shows abnormal intrahepatic ductal arborization, with foci of stricture, dilation, and an abnormally abrupt termination of ducts image. Note the grossly dilated CBD with irregular ductal lining and a strictured distal duct image.
image
Frontal ERCP in a young man with AIDS and signs of cholangitis shows irregular dilation of the intrahepatic and extrahepatic bile ducts, probably due to a distal CBD stricture. Note the abnormal biliary arborization and pneumobilia image.
image
An oblique ERCP in a 30-year-old man with AIDS presenting with a fever, abdominal pain, and abnormal liver function shows evidence of biliary strictures and papillary stenosis. Note the stricture of the distal common bile duct image.

SELECTED REFERENCES

1. Katabathina, VS, et al. Adult bile duct strictures: role of MR imaging and MR cholangiopancreatography in characterization. Radiographics. 2014; 34(3):565–586.

 

Imam, MH, et al. Secondary sclerosing cholangitis: pathogenesis, diagnosis, and management. Clin Liver Dis. 2013; 17(2):269–277.

Tonolini, M, et al. HIV-related/AIDS cholangiopathy: pictorial review with emphasis on MRCP findings and differential diagnosis. Clin Imaging. 2013; 37(2):219–226.

Azizi, L, et al. MR Imaging of sclerosing cholangitis. Clin Res Hepatol Gastroenterol. 2012; 36(2):130–138.

Devarbhavi, H, et al. HIV/AIDS cholangiopathy: clinical spectrum, cholangiographic features and outcome in 30 patients. J Gastroenterol Hepatol. 2010; 25(10):1656–1660.

Vermani, N, et al. MR cholangiopancreatographic demonstration of biliary tract abnormalities in AIDS cholangiopathy: report of two cases. Clin Radiol. 2009; 64(3):335–338.

Bilgin, M, et al. Hepatobiliary and pancreatic MRI and MRCP findings in patients with HIV infection. AJR Am J Roentgenol. 2008; 191(1):228–232.

Knowlton, JQ, et al. Imaging of biliary tract inflammation: an update. AJR Am J Roentgenol. 2008; 190(4):984–992.

Yusuf, TE, et al. AIDS Cholangiopathy. Curr Treat Options Gastroenterol. 2004; 7(2):111–117.

Enns, R. AIDS cholangiopathy: “an endangered disease”. Am J Gastroenterol. 2003; 98(10):2111–2112.

Ko, WF, et al. Prognostic factors for the survival of patients with AIDS cholangiopathy. Am J Gastroenterol. 2003; 98(10):2176–2181.

Chen, XM, et al. Cryptosporidiosis and the pathogenesis of AIDS-cholangiopathy. Semin Liver Dis. 2002; 22(3):277–289.

Mukhopadhyay, S, et al. AIDS cholangiopathy as initial presentation of HIV infection. Trop Gastroenterol. 2001; 22(1):29–30.

Mahajani, RV, et al. Cholangiopathy in HIV-infected patients. Clin Liver Dis. 1999; 3(3):669–684. [x].

Cello, JP. AIDS-Related biliary tract disease. Gastrointest Endosc Clin N Am. 1998; 8(4):963.

Wilcox, CM, et al. Hepatobiliary diseases in patients with AIDS: focus on AIDS cholangiopathy and gallbladder disease. Dig Dis. 1998; 16(4):205–213.

Castiella, A, et al. Ursodeoxycholic acid in the treatment of AIDS-associated cholangiopathy. Am J Med. 1997; 103(2):170–171.

Lefkowitch, JH. The liver in AIDS. Semin Liver Dis. 1997; 17(4):335–344.

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