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Communications to the Editor |

Zafirlukast and Churg-Strauss Syndrome FREE TO VIEW

Michael E. Wechsler, MD; Jeffrey M. Drazen, MD, FCCP (Brigham & Women’s Hospital, Boston, MA)
Author and Funding Information

Dr. Drazen has served (within the past 5 years) as a consultant to the following pharmaceutical companies: Abbott, Bayer, Biogen, Eli Lilly, Forest Laboratories, Genetics Institute, Genome Therapeutics, Glaxo, Marion-Merrill-Dow, Merck, Pfizer, Roche Bioscience, Schering, Sepracor, and Zeneca. Dr. Drazen’s laboratory has received support for asthma trials from Abbott, Astra, Genetics Institute, Immunologics, Merck, Millenium, Schering, Wyeth-Ayerst, and Zeneca. Dr. Drazen holds no equity position in any of these entities. He serves on the scientific advisory board of HiLife Health Systems, Inspire Pharmaceuticals, and Aradigm Medical Products. As compensation for service on these boards, Dr. Drazen holds equity positions in each of these entities.

Correspondence to: Michael E. Wechsler, MD, Division of Pulmonary and Critical Care, Brigham & Women’s Hospital, 75 Francis St., Boston, MA 02115; e-mail: mwechsler@partners.org



Chest. 1999;116(1):266-267. doi:10.1378/chest.116.1.266-a
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Published online

To the Editor:

The case report by Knoell et al (July 1998)1of a patient with Churg-Strauss syndrome associated with zafirlukast is very similar to the case we reported in JAMA in February 19982 of eight patients who developed a similar syndrome. In all of the cases, the patients developed at least four of the six criteria required for patients to be considered as having the Churg-Strauss syndrome in association with zafirlukast. In their case report, within 1 month of taking zafirlukast for worsening asthma, the patient developed a biopsy-proven eosinophilic vasculitic rash, pulmonary infiltrates, and extravascular eosinophils on lung biopsy. Unlike the cases reported in our series, Knoell et al1 found no evidence of cardiomyopathy in their patients.

The authors contrast their case report with our series by stating that all of our cases occurred in the setting of corticosteroid withdrawal. However, their patient had “experienced multiple asthma exacerbations that required treatment with prednisone” and subsequently had been maintained on inhaled corticosteroids when the syndrome occurred. It is our contention that this patient’s severe asthma that required frequent courses of corticosteroids was the heralding event of incipient Churg-Strauss syndrome. We believe that the corticosteroids that were given to treat severe asthma likely masked the development of other systemic eosinophilic manifestations that occurred subsequently. Even the inhaled steroids that the patient was receiving could have masked the syndrome.3While there was an association with zafirlukast use, there is still no convincing evidence that the medication can be causally linked because the patient’s course remains consistent with the natural course of the Churg-Strauss syndrome (ie, indolent allergic disease that evolves into asthma with multiple exacerbations that may progress to eosinophilia and then finally progress to multiorgan systemic eosinophilic vasculitis).4 We agree that health care providers must remain wary of the syndrome, especially in subjects with steroid-dependent asthma, but we also feel strongly that all new cases should be reported to the drug’s manufacturer and to the US Food and Drug Administration so that proper assessment of disease pathogenesis can occur.

References

Knoell, DL, Lucas, J, Allen, JN (1998) Churg-Strauss syndrome associated with zafirlukast.Chest114,332-334. [CrossRef]
 
Wechsler, ME, Garpestad, E, Flier, SR, et al Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast.JAMA1998;279,455-457. [CrossRef]
 
Churg, A, Brallas, M, Cronin, SR, et al Formes frustes of Churg-Strauss syndrome.Chest1995;108,320-323. [CrossRef]
 
Lanham, JG, Elkon, KB, Pusey, CD, et al Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.Medicine (Baltimore)1983;63,65-81
 

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References

Knoell, DL, Lucas, J, Allen, JN (1998) Churg-Strauss syndrome associated with zafirlukast.Chest114,332-334. [CrossRef]
 
Wechsler, ME, Garpestad, E, Flier, SR, et al Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast.JAMA1998;279,455-457. [CrossRef]
 
Churg, A, Brallas, M, Cronin, SR, et al Formes frustes of Churg-Strauss syndrome.Chest1995;108,320-323. [CrossRef]
 
Lanham, JG, Elkon, KB, Pusey, CD, et al Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.Medicine (Baltimore)1983;63,65-81
 
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