Churg-Strauss syndrome medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
Eosinophilic granulomatosis with polyangiitis responds well to treatment with glucocorticoids such as [[prednisone]] when the disease is not in a life threatening state. Patients who present with a life threatening form, are treated with cyclophosphamide together with a glucocorticoid. | |||
==Medical Therapy== | ==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 1 mg/kg/day for 2-3 weeks. Patients who are unable to taper the dose of glucocorticoid less than 7.5 mg/day for 3 to 4 months. Patients not responding to tapered doses can be given a glucocorticoid-sparing agent. These agents include [[methotrexate]],[[azathioprine]],[[hydroxyurea]], and meclophenalate mofetil.<ref name="pmid15030353">{{cite journal| author=Assaf C, Mewis G, Orfanos CE, Geilen CC| title=Churg-Strauss syndrome: successful treatment with mycophenolate mofetil. | journal=Br J Dermatol | year= 2004 | volume= 150 | issue= 3 | pages= 598-600 | pmid=15030353 | doi=10.1111/j.1365-2133.2003.05807.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15030353 }}</ref> <ref name="pmid25971154">{{cite journal| author=Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V et al.| title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. | journal=Eur J Intern Med | year= 2015 | volume= 26 | issue= 7 | pages= 545-53 | pmid=25971154 | doi=10.1016/j.ejim.2015.04.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25971154 }}</ref> <ref name="pmid259711543">{{cite journal| author=Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V et al.| title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. | journal=Eur J Intern Med | year= 2015 | volume= 26 | issue= 7 | pages= 545-53 | pmid=25971154 | doi=10.1016/j.ejim.2015.04.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25971154 }}</ref> | 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 1 mg/kg/day for 2-3 weeks. Patients who are unable to taper the dose of glucocorticoid less than 7.5 mg/day for 3 to 4 months. Patients not responding to tapered doses can be given a glucocorticoid-sparing agent. These agents include [[methotrexate]],[[azathioprine]],[[hydroxyurea]], and meclophenalate mofetil.<ref name="pmid15030353">{{cite journal| author=Assaf C, Mewis G, Orfanos CE, Geilen CC| title=Churg-Strauss syndrome: successful treatment with mycophenolate mofetil. | journal=Br J Dermatol | year= 2004 | volume= 150 | issue= 3 | pages= 598-600 | pmid=15030353 | doi=10.1111/j.1365-2133.2003.05807.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15030353 }}</ref> <ref name="pmid25971154">{{cite journal| author=Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V et al.| title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. | journal=Eur J Intern Med | year= 2015 | volume= 26 | issue= 7 | pages= 545-53 | pmid=25971154 | doi=10.1016/j.ejim.2015.04.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25971154 }}</ref> <ref name="pmid259711543">{{cite journal| author=Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V et al.| title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. | journal=Eur J Intern Med | year= 2015 | volume= 26 | issue= 7 | pages= 545-53 | pmid=25971154 | doi=10.1016/j.ejim.2015.04.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25971154 }}</ref> | ||
Patients who present with a life | Patients who present with a life threatening form, are treated with [[cyclophosphamide]] together with a 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, after which cyclophosphamide may be infused every 3 weeks at 15 mg/kg. Cyclophosphamide infusion doses should not exceed 1.2 g. Patients generally 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: | 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: | ||
Revision as of 12:52, 23 November 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]
Overview
Eosinophilic granulomatosis with polyangiitis responds well to treatment with glucocorticoids such as prednisone when the disease is not in a life threatening state. Patients who present with a life threatening form, are treated with cyclophosphamide together with a glucocorticoid.
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 1 mg/kg/day for 2-3 weeks. Patients who are unable to taper the dose of glucocorticoid less than 7.5 mg/day for 3 to 4 months. Patients not responding to tapered doses can be given a glucocorticoid-sparing agent. These agents include methotrexate,azathioprine,hydroxyurea, and meclophenalate mofetil.[1] [2] [3]
Patients who present with a life threatening form, are treated with cyclophosphamide together with a 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, after which cyclophosphamide may be infused every 3 weeks at 15 mg/kg. Cyclophosphamide infusion doses should not exceed 1.2 g. Patients generally 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 or 2 to 3 weeks after pulse administrative doses. The time frame of how long these medications can be administered for is currently unknown; however, 18-24 months of remission have been noted between both of these medications.[4] According to Sanders et al, 2005 at 18 months the relapse rate when using azathioprine was 13.3% and after 5 years the relapse rate was 62.3%.[5]
Rituximab
Currently rituximab in patients with Eosinophilic granulomatosis with polyangiitis in not licensed for use and studies are required to test for its effectiveness.
Intravenous Immunoglobulins
In Eosinophilic granulomatosis with polyangiitis IV immungoglobulins, are used as a second-line therapy. They are administered with glucocorticoids or together with glucocorticoids + immunosuppressive agents, to patients who experience flare-ups due to certain medications they take or during pregnancy.
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.
- ↑ 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.