The microsporidian Encephalitozoon hellem is being reported with incre
asing frequency in HIV-positive subjects, as an agent of disseminated
microsporidiosis without involving the gastrointestinal tract. We desc
ribe a case of pulmonary microsporidiosis in a 27-year-old Italian man
with AIDS who developed fever, cough, and dyspnea. A chest X-ray show
ed multiple bilateral pulmonary opacities and mediastinal lymph-node e
nlargement. Stained smears of bronchoalveolar lavage sediment showed o
val structures consistent with microsporidian spores. Viral, bacterial
and fungal cultures were repeatedly negative, whereas microsporidia w
ere successfully cultured in human and bovine fibroblast cell lines. A
nalysis of electron micrographs indicated that the isolate belonged to
the genus Encephalitozoon. Based on further immunological, biochemica
l and molecular studies it was characterized as E. hellem. Even though
a temporary improvement with albendazole therapy was noticed the pati
ent deteriorated clinically and died of severe respiratory distress.