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 in a life threatening state. The dose of glucocorticoid that is given is 7.5 mg/day for 3 to 4 months. After this period, the dose of glucocorticoids in tapered down. However, that all patients respond well when the dose of glucocorticoid is tapered below 7.5 mg/day. Patients not responding well can be given a glucocorticoid-sparing agent along with there glucocorticoid. These agents include methotrexate, azathioprine, hydroxyurea, and meclophenalate mofetil.[1] [2]
Patients who present with a life threating form of the disease are treated with Cyclophosphamide together with glucocorticoid. The dosage that is given is 1 mg/kg/day for 2-3 weeks and then slowly tapered to a minimal effective dose. Cyclophosphamide is dosed at 2mg/kg/day as an oral or intravenous dose. Cyclophosphamide may also be administered via pulse infusions at 15mg/kg every 2 weeks for the first three infusions. Following the 3 infusions of cyclophosphamide, cyclophosphamide is infused at 15 mg/kg every 3 weeks. Infused doses of cyclophosphamide should not exceed doses of 1.2 g. Patients receive 6 to 12 pulse infusions of cyclophosphamide.
The maintenance therapy of Eosinophilic granulomatosis with polyangiitis that presents as life threating, patients are treated with Azathioprine or Methotrexate. Treatment with either of these two agents is given as follows:
| Maintenance therapy | Azathioprine | Methotrexate |
|---|---|---|
| 2 mg/kg/day | 10-30 mg/wk |
Either of these treatments can be given after a few days of oral cyclophosphamide; to 2 to 3 weeks after pulse administrative doses. The time frame at which these medications are to be administered to is currently unknown; however, 18-24 months of remission of these 2 agents have been noted.[3]
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.[4]
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
- ↑ Assaf C, Mewis G, Orfanos CE, Geilen CC (2004). "Churg-Strauss syndrome: successful treatment with mycophenolate mofetil". Br J Dermatol. 150 (3): 598–600. doi:10.1111/j.1365-2133.2003.05807.x. PMID 15030353.
- ↑ Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V; et al. (2015). "Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management". Eur J Intern Med. 26 (7): 545–53. doi:10.1016/j.ejim.2015.04.022. PMID 25971154.
- ↑ Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V; et al. (2015). "Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management". Eur J Intern Med. 26 (7): 545–53. doi:10.1016/j.ejim.2015.04.022. PMID 25971154.
- ↑ 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.