Invasive aspergillosis is seldomly described in systemic lupus erythematosus. We present two cases of aspergillosis and review 21 cases reported between 1957 and 1994. The typical clinical presentation is fever and cough in a hospitalized SLE patient previously treated with corticosteroids, immunosuppressors, and broad-spectrum antibiotics. Unlike aspergillosis in other conditions, granulocytopenia is uncommon. Chest radiographs show diffuse or patchy infiltration of lung fields. Diagnosis was suspected premortem in 2 patients. Aspergillus fumigatus was identified or isolated in sputum or parenchimal tissues in the majority of cases. Twenty-two patients died (95%). The finding of hyphae in the sputum of a systemic lupus erythematosus patient with a suggestive clinical picture should lead to bronchoscopy, bronchoalveolar lavage, and lung biopsy. Proof of diagnosis will come from the demonstration of hyphae in tissues and isolation of aspergillus from tissue cultures. Long-term therapy with amphotericin B alone or in combination with fluorocytosine or itraconazole may help improve survival.