Correspondence
Case 30-2000: Churg–Strauss Syndrome
N Engl J Med 2001; 344:858-859March 15, 2001
- Article
To the Editor:
Dr. Arm's discussion (Sept. 28 issue)1 of a case of the Churg–Strauss syndrome in a man who was receiving multiple asthma medications, including montelukast (Singulair, Merck), did not include important medical history. Information received through our pharmacovigilance program indicates that, before treatment with montelukast was started, the patient had recurrent bilateral pulmonary infiltrates and an ill-defined episode of asthma, diarrhea, weight loss, anemia, and pleural effusion, which responded rapidly to treatment with high doses of corticosteroids. All this suggests that the patient had a forme fruste of the Churg–Strauss syndrome before the initiation of montelukast.
Elements of the Churg–Strauss syndrome (Table 1Table 1
Prior Manifestations of the Churg–Strauss Syndrome in Patients Who Were Given a Diagnosis of the Syndrome after Therapy with Montelukast.), in addition to asthma, were observed before the initiation of montelukast treatment in 14 of the first 16 reports to Merck of the Churg–Strauss syndrome in association with montelukast use. As in the case discussed by Arm, this sequence appears to rule out montelukast as a primary cause of the Churg–Strauss syndrome in these cases. Corticosteroids were withdrawn or reduced before the diagnosis in 15 of the 16 reports. As suggested by Wechsler et al.,2 a reduction in corticosteroids, which might be facilitated by the use of a leukotriene modifier, may play a decisive part in the appearance of the Churg–Strauss syndrome.The patient described in the Case Records was also included (as Case 2) in a report by Wechsler et al.3
3 ReferencesPeter Gruer, M.B., Ch.B.
Thomas Bold, M.D.
Laura Vilardo, M.S.
Merck Research Laboratories, West Point, PA 194861
Case Records of the Massachusetts General Hospital (Case 30-2000). N Engl J Med 2000;343:953-961
Full Text | Web of Science | Medline2
Wechsler ME ,Pauwels R ,Drazen JM . Leukotriene modifiers and Churg-Strauss syndrome: adverse effect or response to corticosteroid withdrawal? Drug Saf 1999;21:241-251
CrossRef | Web of Science | Medline3
Wechsler ME ,Finn D ,Gunawardena D , et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest 2000;117:708-713
CrossRef | Web of Science | Medline
Author/Editor Response
Dr. Arm replies:
To the Editor: I am grateful to Gruer and colleagues for their comments on this case. The additional history they provide was not available to me at the time of the case presentation. The apparent association of the introduction of a new asthma therapy with the onset of the Churg–Strauss syndrome has been observed for medications other than leukotriene-modifying drugs, such as inhaled corticosteroids.1 Wechsler has suggested that this apparent association may be due to the withdrawal of corticosteroids or may reflect the natural history of a disease in which new therapy has been introduced in the presence of worsening asthma.2 The pathobiology and epidemiology of the Churg–Strauss syndrome are poorly understood, making it difficult to comment on the possible role of certain asthma therapies in facilitating the onset or progression of the syndrome in a subgroup of patients. The Churg–Strauss syndrome is a rare disease, and modern asthma therapy, including low-to-moderate doses of inhaled corticosteroids and leukotriene-modifying drugs, provides effective and safe treatment in the majority of patients. When oral corticosteroids are withdrawn, patients should be carefully monitored for evidence of systemic disease.
2 ReferencesJonathan P. Arm, M.D.
Brigham and Women's Hospital, Boston, MA 021151
Wechsler ME ,Finn D ,Gunawardena D , et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest 2000;117:708-713
CrossRef | Web of Science | Medline2
Le Gall C ,Pham S ,Vignes S , et al. Inhaled corticosteroids and Churg-Strauss syndrome: a report of five cases. Eur Respir J 2000;15:978-981
CrossRef | Web of Science | Medline
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