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Diagnostic Ultrasound - Abdomen and Pelvis

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AIDS-Related Cholangiopathy<br />

TERMINOLOGY<br />

Abbreviations<br />

• AIDS cholangitis, AIDS-related sclerosing cholangitis, AIDS<br />

cholangiopathy, HIV cholangiopathy<br />

Definitions<br />

• Secondary sclerosing cholangitis usually resulting from<br />

opportunistic infection of biliary tract in AIDS patients with<br />

CD4 count < 100/mm³<br />

IMAGING<br />

General Features<br />

• Best diagnostic clue<br />

○ AIDS patient with papillary stenosis, intrahepatic<br />

strictures, thickened bile ducts <strong>and</strong> gallbladder (GB)<br />

Ultrasonographic Findings<br />

• Grayscale ultrasound<br />

○ Focal biliary duct strictures <strong>and</strong> associated dilatation<br />

○ Bile duct wall thickening<br />

○ Dilatation <strong>and</strong> wall thickening of common bile duct<br />

(CBD) with papillary stenosis<br />

○ Diffuse GB wall thickening without gallstones<br />

Radiographic Findings<br />

• ERCP<br />

○ Sclerosing cholangitis <strong>and</strong> papillary stenosis<br />

– Unique to AIDS cholangiopathy<br />

○ Long extrahepatic bile duct strictures, ±intrahepatic<br />

sclerosing cholangitis<br />

MR Findings<br />

• MRCP<br />

○ Multiple intra- <strong>and</strong> extrahepatic biliary strictures, with<br />

associated dilation<br />

○ Papillary stenosis with CBD dilation<br />

○ Isolated intermediate to long segment CBD stricture<br />

○ GB wall <strong>and</strong> bile duct thickening<br />

Imaging Recommendations<br />

• Best imaging tool<br />

○ US: Initial imaging test<br />

– Normal US essentially rules out diagnosis<br />

○ MRCP<br />

○ ERCP: <strong>Diagnostic</strong> <strong>and</strong> therapeutic<br />

DIFFERENTIAL DIAGNOSIS<br />

Primary Sclerosing Cholangitis<br />

• Multifocal irregular ductal strictures with alternating ductal<br />

ectasia<br />

• Affects intrahepatic <strong>and</strong> extrahepatic ducts<br />

Autoimmune Cholangitis<br />

• Bile duct wall thickening <strong>and</strong> strictures<br />

• Most commonly affects distal CBD, can affect intrahepatic<br />

ducts<br />

• Seen in setting of autoimmune pancreatitis<br />

Ascending Cholangitis (Acute Bacterial Cholangitis)<br />

• Ductal stricture <strong>and</strong> biliary wall thickening<br />

• Usually secondary to choledocholithiasis<br />

Cholangiocarcinoma<br />

• Infiltrative mass along ductal epithelium<br />

• Invades hepatic parenchyma <strong>and</strong> regional lymph node<br />

metastases<br />

PATHOLOGY<br />

General Features<br />

• Chronic inflammation of biliary tract from opportunistic<br />

pathogens<br />

• Most common pathogens: Cryptosporidium, CMV<br />

CLINICAL ISSUES<br />

Presentation<br />

• Most common signs/symptoms<br />

○ Epigastric/RUQ pain, diarrhea<br />

○ Fever <strong>and</strong> jaundice, less common<br />

• Clinical profile<br />

○ ↑ ↑ cholestatic enzymes (Alk Phos <strong>and</strong> GGT)<br />

○ Mild ↑ AST/ALT, bilirubin normal or↑<br />

Demographics<br />

• Epidemiology<br />

○ Late-stage AIDS patients (CD4 < 100/mm³)<br />

○ ↓ ↓ incidence 2° highly active retroviral therapy (HAART)<br />

Natural History & Prognosis<br />

• Survival not affected by cholangiopathy<br />

• Mortality rate determined by natural history of AIDS<br />

Treatment<br />

• Papillary stenosis with symptoms: Sphincterotomy for pain<br />

relief, does not alter intrahepatic disease<br />

• Dominant CBD stricture: Stenting<br />

• Intra- or extrahepatic sclerosing cholangitis: Options<br />

limited, mainstay is restoration of immune system with<br />

HAART<br />

DIAGNOSTIC CHECKLIST<br />

Image Interpretation Pearls<br />

• AIDS patient with papillary stenosis, intrahepatic strictures,<br />

or acalculous cholecystitis<br />

SELECTED REFERENCES<br />

1. Katabathina VS et al: Adult bile duct strictures: role of MR imaging <strong>and</strong> MR<br />

cholangiopancreatography in characterization. Radiographics. 34(3):565-86,<br />

2014<br />

2. Datta J et al: Extrahepatic cholangiocarcinoma developing in the setting of<br />

AIDS cholangiopathy. Am Surg. 79(3):321-2, 2013<br />

3. Imam MH et al: Secondary sclerosing cholangitis: pathogenesis, diagnosis,<br />

<strong>and</strong> management. Clin Liver Dis. 17(2):269-77, 2013<br />

4. Tonolini M et al: HIV-related/AIDS cholangiopathy: pictorial review with<br />

emphasis on MRCP findings <strong>and</strong> differential diagnosis. Clin Imaging.<br />

37(2):219-26, 2013<br />

5. Shanbhogue AK et al: Benign biliary strictures: a current comprehensive<br />

clinical <strong>and</strong> imaging review. AJR Am J Roentgenol. 197(2):W295-306, 2011<br />

6. Catalano OA et al: Biliary infections: spectrum of imaging findings <strong>and</strong><br />

management. Radiographics. 29(7):2059-80, 2009<br />

7. Daly CA et al: Sonographic prediction of a normal or abnormal ERCP in<br />

suspected AIDS related sclerosing cholangitis. Clin Radiol. 51(9):618-21, 1996<br />

8. Lack, E. E. Pathology of the Pancreas, Gallbladder, Extrahepatic Biliary Tract,<br />

<strong>and</strong> Ampullary Region. Oxford University Press. 2003<br />

Diagnoses: Biliary System<br />

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