Microsporidia were recognized as causing disease in animals as early as the 1920s but were not recognized as agents of human disease until the AIDS pandemic began in the mid-1980s. Before then, several earlier human cases had been reported but were thought to be very unusual.

Enterocytozoon bieneusi

A number of cases of E. bieneusi infection have been reported in patients with AIDS. Chronic intractable diarrhea, fever, malaise, and weight loss are symptoms of E. bieneusi infections, and these symptoms mimic those seen with cryptosporidiosis or isosporiasis. Often these patients have four to eight watery, nonbloody stools each day, accompanied by nausea and anorexia. Dehydration and D-xylose and fat malabsorption also may develop. These patients tend to be severely immunodeficient, with a CD4 count almost always below 200 cells/mm3 and often below 100 cells/mm3. Mixed infections with E. bieneusi and E. intestinalis have also been reported. E. bieneusi infection has been implicated in AIDS-related sclerosing cholangitis. However, demonstration of E. bieneusi spores in extraepithelial tissues does not always appear to be associated with subsequent development of systemic infection.

E. bieneusi spores have been identified in sputum and bronchoalveolar lavage fluid in addition to stool specimens. E. bieneusi can colonize the respiratory tract, and clinical specimens from these specimens may reveal the presence of spores. Multiorgan microsporidiosis caused by E. bieneusi has been diagnosed in patients infected with HIV; organisms have been recovered in stools, duodenal biopsy specimens, nasal discharge, and sputum.

Infection with E. bieneusi has also been reported in immunocompetent individuals; symptoms were self limited, and diarrheal disease resolved within 2 weeks. E. bieneusi may be more commonly associated with sporadic diarrheal disease than was previously suspected, and the immune system may play a role in the control of this intestinal infection. It is also quite possible that E. bieneusi may persist as an asymptomatic infection in immunocompetent individuals.

Encephalitozoon spp.

 Both Encephalitozoon cuniculi and Encephalitozoon hellem have been isolated from human infections. The spectrum of disease in patients with AIDS, organ transplant recipients, and otherwise immunocompromised patients includes keratoconjunctivitis, intraocular infection, sinusitis, bronchiolitis, pneumonitis, nephritis, ureteritis, cystitis, prostatitis, urethritis, hepatitis, sclerosing cholangitis, peritonitis, diarrhea, and encephalitis. Clinical manifestations may vary, ranging from an asymptomatic carrier state to organ failure.

Encephalitozoon (Septata) intestinalis

 Encephalitozoon (Septata) intestinalis infects primarily small intestinal enterocytes, but infection does not remain con fined to epithelial cells. E. intestinalis is also found in lamina propria macrophages, fibroblasts, and endothelial cells. Dissemination to the kidneys, lower airways, and biliary tract appears to occur through infected macro phages. Fortunately, these infections tend to respond to therapy with albendazole, unlike infections caused by E. bieneusi.

Other Microsporidia

Different microsporidial species have been isolated from immunocompetent individuals who presented with keratoconjunctivitis, severe keratitis, or corneal ulcers. Also, keratoconjunctivitis has been found in an immunocom petent contact lens wearer.

In immunocompromised patients, myositis has been seen in infections caused by Pleistophora sp., Pleistophora ronneafiei, Trichomonas hominis, Anncaliia vesicularum, and Anncaliia algerae. Trachipleistophora anthropophthera has been identified at autopsy in cerebral, cardiac, renal, pancreatic, thyroid, hepatic, splenic, lymphoid, and bone marrow tissue of patients with AIDS. Disseminated infection caused by Anncaliia connori was found at autopsy in a 4-month-old athymic male infant.

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