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Microsporidial AIDS cholangiopathy due to Encephalitozoon intestinalis: case report and review

R A Sheikh1, T P Prindiville, S Yenamandra

  • 1Department of Internal Medicine, University of California Davis, Sacramento 95932, USA.

The American Journal of Gastroenterology
|September 28, 2000
PubMed

Insights

Encephalitozoon intestinalis, a microsporidian, caused AIDS cholangiopathy in an immunocompromised patient. Early albendazole treatment improved symptoms and CD4 count, but bile duct damage persisted.

Area of Science:

  • Medical Mycology
  • Gastroenterology
  • Infectious Diseases

Background:

  • Microsporidia are opportunistic pathogens, frequently affecting immunocompromised individuals, particularly those with Acquired Immunodeficiency Syndrome (AIDS).
  • Enterocytozoon bieneusi and Encephalitozoon intestinalis are the primary microsporidian species causing disease in AIDS patients, with diarrhea being the most common symptom.
  • Encephalitozoon intestinalis has been implicated in disseminated infections, including cholangitis, a condition affecting the bile ducts.

Observation:

  • A case of AIDS cholangiopathy presented with abdominal pain and cholestatic liver tests, mimicking sclerosing cholangitis.
  • Initial diagnostic tests, including bile and stool samples, were negative for microsporidia. Routine microscopy of biopsy specimens also failed to identify the organism.
  • Encephalitozoon intestinalis spores were definitively identified in bile duct biopsies using specialized staining and electron microscopy.

Findings:

  • Albendazole therapy successfully eradicated Encephalitozoon intestinalis, leading to clinical improvement and an increased CD4 count.
  • Despite successful infection eradication, the cholangiographic findings showed progressive bile duct injury, indicating irreversible damage.
  • The delay in albendazole administration, requiring compassionate use approval, may have contributed to the persistent bile duct damage.

Implications:

  • This case highlights Encephalitozoon intestinalis as an unusual cause of sclerosing cholangitis in AIDS patients.
  • Early diagnosis and prompt treatment with albendazole are crucial for managing microsporidial infections, though they may not reverse established biliary damage.
  • Management of such cases may necessitate interventions like biliary sphincterotomy and stent placement to address progressive bile duct stricturing.

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