Study objectives: We previously reported eight patients
who developed Churg-Strauss syndrome in association with zafirlukast
treatment for asthma and postulated that the syndrome resulted from
unmasking of a previously existing condition due to corticosteroid
withdrawal and not from a direct drug effect. The availability of
montelukast, a new leukotriene receptor antagonist with a different
molecular structure, permitted us to test this hypothesis. Our goals
were to ascertain whether the Churg-Strauss syndrome developed in
patients taking montelukast and other novel asthma medications, and to
describe potential mechanisms for the syndrome.
Design: Case series.
and hospital practices of pulmonologists in the United States and
Patients: Four adults (one man, three
women) who received montelukast as treatment for asthma; two women who
received salmeterol/fluticasone therapy, but not montelukast.
Results: Churg-Strauss syndrome developed in the four
asthmatic patients who received montelukast. In each case, there was a
long history of difficult-to-control asthma characterized by multiple
exacerbations that had required frequent courses of oral systemic
corticosteroids or high doses of inhaled corticosteroids for control.
Two other asthmatics who received fluticasone and salmeterol but not
montelukast therapy developed the same syndrome with tapering doses of
oral or high doses of inhaled corticosteroids.
Conclusions: The occurrence of Churg-Strauss syndrome in
asthmatic patients receiving leukotriene modifiers appears to be
related to unmasking of an underlying vasculitic syndrome that is
initially clinically recognized as moderate to severe asthma and
treated with corticosteroids. Montelukast does not appear to directly
cause the syndrome in these patients.