Churg-Strauss syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Medical Therapy

Eosinophilic granulomatosis with polyangiitis responds well to treatment with glucocorticoids such as prednisone when the disease is not severe. The dosage that is given is 1 mg/kg/day for 2-3 weeks and then slowly tapered to a minimal effective dose. Patients

Treatment for Churg-Strauss syndrome includes glucocorticoids such as prednisone at a dose of 0.5-1.5 mg/kg per day x 6-12 weeks and other immunosupressive drugs such as azathioprine and cyclophosphamide. In many cases the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and life long.

A systematic review conducted in 2007 indicated that all patients should be treated with high-dose steroids, but that in patients with an FFS of 1 or higher cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to less relapses than 6. Remission can be maintained with a less toxic drug, such as azathioprine or methotrexate.[1]

The erythrocyte sedimentation rate (ESR) and eosinophil count can be followed to gauge the response to therapy. Late relapses are uncommon and refractory disease may require Cyclophosphamide, Azathioprine, intravenous immunoglobulin (IVIG) and plasmapheresis.

References

  1. Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA. 298 (6): 655–69. doi:10.1001/jama.298.6.655. PMID 17684188.

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