Churg-Strauss syndrome treatment

Zafirlukast and montelukast are well tolerated. Zafirlukast increases plasma concentrations of warfarin and decreases the concentrations of theophylline and erythromycin. In rare cases, treatment of patients with CysLT receptor antagonists is associated with the development of Churg-Strauss syndrome, a condition marked by acute vasculitis, eosinophilia, and a worsening of pulmonary symptoms. Because these symptoms often appear when patients are given the leukotriene receptor antagonists when they are being weaned from oral corticosteroid therapy, it is not clear whether they are related to the action of the antagonists or are due to a sudden reduction in corticosteroid therapy. 

Of these agents, zileuton is the least prescribed because of reports of occasional liver toxicity. The receptor antagonists appear to have little toxicity. Reports of Churg-Strauss syndrome (a systemic vasculitis accompanied by worsening asthma, pulmonary infiltrates, and eosinophilia) appear to have been coincidental, with the syndrome unmasked by the reduction in prednisone dosage made possible by the addition of zafirlukast or montelukast. Of these two, montelukast is the most prescribed, probably because it can be taken without regard to meals and because of the convenience of once-daily treatment. 

The authors of the second report commented that activated eosinophils and their cytotoxic products, such as eosinophil catatonic protein, may play a part in the pathogenesis of Churg-Strauss syndrome. Measuring serum concentrations of eosinophil catatonic protein may be useful in monitoring disease activity, since concentrations were increased before treatment and normalized afterwards. 

Eight patients with steroid-dependent asthma, who had been able to either discontinue or reduce their oral glucocorticoid requirement subsequent to starting treatment with zafirlukast, developed Churg-Strauss syndrome (7). 

A 52-year-old woman developed Churg-Strauss syndrome some 12 weeks after beginning treatment with pranlukast and 8 weeks after discontinuing low-dose oral prednisolone (5 mg on alternate days) (17). 

Churg-Strauss syndrome has been reported with montelukast in a patient who had not taken oral glucocorticoids. Symptoms developed within 2 days of beginning treatment with montelukast (18). 

Green RL, Vayonis AG. Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment. Lancet 1999 353(9154) 725-6. 

A 26-year-old asthmatic woman had severe acute necrotizing eosinophilic endomyocarditis while taking pranlukast, inhaled beclomethasone, and oral theophylline (4). Oral prednisolone had been replaced by pranlukast 9 months before the event. Cardiac injury was accompanied by peripheral eosinophilia, cardiogenic shock, and pulmonary infiltrates, suggesting atypical Churg-Strauss syndrome. She recovered after intensive treatment, steroid pulse therapy, and withdrawal of pranlukast. 

Glucocorticoids are used widely in the treatment of a variety of rheumatic disorders and are a mainstay in the treatment of the more serious inflammatory rheumatic diseases, such as systemic lupus erythematosus, and a variety of vasculitic disorders, such as polyarteritis nodosa, Wegener s granulomatosis, Churg-Strauss syndrome, and giant cell arteritis. 

Tertneer CC, Simon JC, Schopf E. Low-dose interferon alfa-2b for the treatment of Churg-Strauss syndrome with prominent skin involvement. Arch Dermatol 2001 137(2) 136-138. 

Tatsis E, Schnahel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med 1998 129(5) 370-374. 

Wechsler ME, Finn D, Gunawardena D, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest 2000 117(3) 708-713. 

The second case was a 62-year old female asthmatic treated with salmeterol/fluticasone combination for the previous 5 years, and with montelukast during the previous 5 months [81 ]. The patient presented with sensory deficits in lower extremities and diffuse musculoskeletal and thoracic pain. She was diagnosed with Churg-Strauss syndrome, and met more than four of the American of Rheumatology criteria for Churg-Strauss. With discontinuation of montelukast and treatment with high dose steroids, the patient s symptoms rapidly improved and eosinophilia decreased within 72 h. 

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