Aspergillus pneumonia in chronic granulomatous disease: recurrence and long-term outcome.

Four of 13 patients followed with chronic granulomatous disease (CGD) developed biopsy-proven Aspergillus pneumonia. Two patients died of disseminated aspergillosis despite intensive treatment; delays in diagnosis appear to have been a major contributing cause. Two patients survived two episodes each of Aspergillus pneumonia, three and six years apart. At 12 and 18 years of age, both are well and active, although their chest X-rays show residual scarring and pulmonary function tests indicate obstructive lung disease. These cases demonstrate that in patients with CGD, 1) multiple episodes of Aspergillus pneumonia can occur; 2) effective immunity to the organism does not develop after infection; and 3) some survivors of Aspergillus pneumonia experience only minimal morbidity.