AIDS cholangiopathy is a biliary syndrome in patients with AIDS. This entity is diagnosed on the basis of clinical features, raised alkaline phosphatase, evidence. AIDS cholangiopathy is a well-documented biliary syndrome in severely immunocompromised AIDS patients. It occurs when strictures in the. As it was suspected that the patient suffered from AIDS cholangiopathy, further laboratory studies were obtained. A stool study for Isospora ova.
|Published (Last):||26 July 2013|
|PDF File Size:||20.84 Mb|
|ePub File Size:||7.59 Mb|
|Price:||Free* [*Free Regsitration Required]|
To receive news and publication updates for International Journal of Hepatology, enter your email address in the box below. This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
AIDS-associated cholangiopathy is cholangiopatht form of biliary tract inflammation with stricture formation seen in AIDS patients who are severely immunosuppressed. It is no longer common in countries in which HAART therapy is widely employed but is still seen in underdeveloped countries. The majority of patients are symptomatic at the time of presentation. Herein, we describe a seventy-four-year-old woman who presented with unilateral leg swelling after a prolonged airplane flight.
AIDS cholangiopathy | Radiology Reference Article |
She was otherwise entirely asymptomatic. Routine laboratory testing was notable for a hypochromic microcytic anemia, slight leukopenia, and mild hypoalbuminemia. Liver enzymes were all elevated. Deep venous thrombosis was confirmed, and a CT scan of the chest disclosed no pulmonary emboli.
However, the visualized portion of the abdomen showed dilatation of the common bile and pancreatic ducts. This was confirmed on ultrasonography and MRCP, and no obstructive lesions were noted. An ERCP revealed a dilated common bile duct without filling defects or strictures.
A balloon occlusion cholangiogram showed strictures and beading of the intrahepatic ducts. A seventy-four-year-old Kenyan woman was admitted to the hospital with bilateral lower extremity edema. Three days earlier, she had arrived to the United States after a sixteen hour flight.
Past medical history included a stroke with no residual deficits and chronic low-back pain. Her family history was unremarkable. The patient had a 30 pack-year history of smoking but had quit 3 years earlier.
She neither drank nor used illicit drugs. She had not been sexually active in more than thirty years. The bilirubin and prothrombin time were normal.
A venous duplex of the lower extremities revealed a thrombus in the left superficial femoral vein extending into the left popliteal vein. She was placed on heparin and warfarin. An initial chest X-ray evealed a right lower lobe patchy infiltrate versus atelectasis.
Given her history of cough and weight loss, the patient was treated with ceftriaxone and azithromycin for possible pneumonia and placed on droplet precautions while a PPD was placed and a QuantiFERON Gold lab test was obtained. Written consent was also obtained to complete an HIV test. A CT scan of the chest showed no significant pulmonary pathology or embolism. The visualized portion of the abdomen showed dilatation of the common bile and pancreatic ducts.
An abdominal ultrasound later confirmed dilatation of the common bile duct and showed central intrahepatic duct dilatation as well. No stones were visualized, but the distal portion of the common bile duct was obscured by overlying bowel gas.
Magnetic resonance cholangiopancreatography MRCP showed pancreatic and biliary ductal dilatation without identification of any obstructive lesions. It was, however, very difficult to fill up the intrahepatic ducts. As it was suspected that the patient suffered from AIDS cholangiopathy, further laboratory studies were obtained. A stool study for Isospora ova and parasites and a direct stool study for Cyclospora were negative.
An electroimmunofluroscent assay for cryptospora was also negative. However, inGuarda et al.
That same year, Pitlik et al. In early studies, AIDS cholangiopathy was estimated to occur in up to 26 percent of AIDS patients, but its true incidence is unknown as many patients are asymptomatic. The etiology of AIDS cholangiopathy is unknown, but it is believed to be related to opportunistic infections in the biliary tract where pathogens have often been detected.
These infections likely cause a secondary sclerosing cholangitis due to the associated continual inflammation. It is theorized that enteric infection leads to portal bacteremia which, in turn, leads to subsequent bile duct injury and destruction [ 8 ]. However, although it has been isolated in the bowel mucosa, it has never been detected in the biliary epithelium.
A genetic predisposition has been postulated as pathogens may trigger an autoimmune-mediated reaction in patients with HLA DRw52a [ 8 ]. This may explain why the features of AIDS cholangiopathy are independent of the associated opportunistic infection [ 9 ]. AIDS cholangiopathy is usually associated with bacterial or Mycobacterium tuberculosis infection when the CD4 count ranges from to cells per cubic mm. With CD4 counts less than cells per cubic mm, associated infections are viral, protozoan or fungal, or caused by Mycobacterium avium intracellulare [ 10 ].
The two most commonly detected pathogens are Cryptosporidium and cytomegalovirus CMV. When there is involvement of the intrahepatic ducts, Cryptosporidium and CMV infection are more likely than when the intrahepatic ducts are not involved [ 10 ]. Cryptosporidium was the first opportunistic infection reported in the bile ducts of AIDS patients in by two independent groups, Pitlik et al.
AIDS patients with CD4 counts less than 50 per cubic millimeter were more likely to have biliary symptoms and an increased risk of death at one year [ 13 ]. Cryptosporidium is associated with severe and diffuse changes in the biliary tract. It initiates an inflammatory response that is mostly composed of lymphocytes with occasional plasma cells and macrophages and rare eosinophils. Neutrophils can be seen in the lumina of dilated glands forming microabscesses.
Epidemiology, determinants, and management of AIDS cholangiopathy: A review
The cells of the biliary epithelium often have enlarged nuclei with an eosinophilic cytoplasm and are transformed into cuboidal or attenuated cells [ 14 ]. Squamous metaplasia can also occur [ 15 ]. Polypoid defects have been hypothesized to be caused by granulation tissue that extends into the bile ducts [ 16 ]. Cryptosporidium parvum has also been linked to the development of sclerosing cholangitis in other immunocompromised patients such as those with X-linked hyper IgM syndrome [ 5 ].
These patients often had coinfection with Cryptosporidium and Candida albicans. Common bile duct dilatation and a mural cuolangiopathy of an acalculous gallbladder were often noted [ 5 ]. It has been suggested that cytomegalovirus could lead to ischemic injury of the biliary tract in a fashion similar to that in posttransplant patients and patients undergoing intraarterial chemotherapy who develop bile duct injury that resembles sclerosing cholangitis.
The inclusion bodies that are cholangioapthy of cytomegalovirus infection are rarely seen in biliary epithelial cells of affected patients, but are frequently found in the arteriole adjacent to the biliary duct [ 17 ]. Often there is an associated lymphocytic infiltrate [ 7 ]. Microsporidia is uncommon in patients with AIDS cholangiopathy.
In a study by Ko et al. It had been suggested that patients with no identified pathogen may have microsporidial infection as multiple biopsies are often required for its detection. Enterocytozoon bieneusi can be detected with hematoxylin and eosin stain, but is more apparent with Giemsa stain. In some cases, electron microscopy may be necessary [ 620 ]. Mycobacterium avium complex is a consideration when one sees poorly formed noncaseating granulomas along with large foamy macrophages and a scarcity of lymphocytes [ 21 ].
Finally, diseases of the pancreas that involve the distal common bile duct can mimic AIDS cholangiopathy [ 21 ]. Abdominal pain is typically more severe when papillary stenosis is present [ 5 ]. It is characteristically sharp in nature and may radiate to the back. Nausea and vomiting are not uncommon [ 22 ]. Fever occurs in over half of patients with AIDS cholangiopathy, and high spiking fevers can indicate a bacterial superinfection [ 7 ].
Patients who are infected with cryptosporidium, microsporidium or MAI additionally may have diarrhea and malabsorption symptoms [ 722 ]. Laboratory examination is helpful but not specific in the diagnosis of AIDS cholangiopathy. The classic laboratory profile in AIDS cholangiopathy is an alkaline phosphatase that is five- to seven-times the upper limit of normal and a moderate increase in transaminase levels. Alkaline phosphatase elevation may be the sole laboratory abnormality in some patients.
Jaundice in AIDS cholangiopathy is usually mild with a total bilirubin less than twice the upper limit of normal indicating incomplete obstruction. Endoscopic ultrasound allows for better detection of dilation and thickening of the common bile duct and can more accurately exclude stones, extrabiliary compression, and tumors when compared to transabdominal ultrasound [ 11 ].
CT is cholanngiopathy useful than ultrasound in identifying intrahepatic strictures [ 7 ]. It is also better at disclosing cholangiopahy of the pancreas and liver [ 9 ]. However, it is less successful than ultrasound in demonstrating wall thickening and strictures of the common bile duct [ 7 ]. Radionuclide hepatobiliary scanning Aics can detect duct dilatation but has been supplanted by other imaging modalities.
It may be a useful in patients suspected aies having acute cholecystitis. Biliary brushings or aspirated biliary fluid may help make the diagnosis of Cryptosporidium or Microsporidia.
It is also particularly useful in patients with severe pain, allowing for sphincterotomy. Four subcategories of AIDS cholangiopathy have been described: It is unknown if cholangiopatgy different subtypes are a continuum of one disease process [ 21 ]. The common bile duct can have a beaded or scalloped appearance. The left intrahepatic ductal system is more severely involved than the right side in most cases.
The combination of papillary stenosis and intrahepatic ductal strictures is relatively unique to AIDS cholangiopathy [ 5 ]. In HIV-infected individuals, the presence of an echogenic nodule in the distal common cbolangiopathy duct may indicate AIDS cholangiopathy, as it is thought to represent edema in the xholangiopathy of Vater [ 25 ].
The intrahepatic findings in AIDS cholangiopathy are comparable to those seen in primary sclerosing cholangitis PSC with a beaded appearance and a diminished arborization or pruning of peripheral branches. Diverticular outpouchings, sacculations, and high-grade proximal strictures are seen in PSC while moderate ductal dilatation with irregular margins and nodules are more characteristic of AIDS cholangiopathy [ 9 ]. Isolated dilatation of intrahepatic biliary ducts in AIDS cholangiopathy i.
In our patient, MRCP showed a dilated common bile duct and subtle irregularities of the intrahepatic ducts. In general, percutaneous liver biopsy has little value in diagnosing AIDS cholangiopathy [ 2126 ].
In addition to imaging features, biopsy will often show an inflammatory infiltrate that is rich in T4 lymphocytes in primary sclersoing cholangitis while these cells are depleted in AIDS patients.