Churg-Strauss syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]
Overview
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
OR
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
OR
The majority of cases of [disease name] are self-limited and require only supportive care.
OR
[Disease name] is a medical emergency and requires prompt treatment.
OR
The mainstay of treatment for [disease name] is [therapy].
OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.
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[Therapy] is recommended among all patients who develop [disease name].
OR
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
OR
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
OR
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Medical Therapy
- Medical therapy for eosinophilic granulomatosis with polyangiitis is according to the guidelines proposed by:[1][2]
- EGPA Consensus Task Force recommendations by EULAR (European League Against Rheumatism)
- American College of Rheumatology (ACR)
- Pharmacologic therapy for EGPA include systemic glucocorticoids(eg, prednisone), immunosupressive agents(eg, cyclophosphamide, azathioprine, methotrexate), inhaled glucocorticoids, IVIG (intravenous immune globulin), anti-IgE(eg, omalizumab), anti-IL-5 antibodies(eg, mepolizumab), and plasma exchange.
- Five factor score(FFS) and Birmingham vasculitis activity score (BVAS) can be used to assess the vasculitis severity and disease activity. These two scoring systems can be helpful in initiating therapy.[3][4]
Systemic glucocorticosteroids
- The first line treatment for all the patients with eosinophilic granulomatosis with polyangiitis are systemic glucocorticoids. Most commonly used drug is prednisone.[1][5][6]
- Preferred regimen:
- For life-threatening vasculitis, intravenous glucocorticoids can be administered.
- Preferred regimen: 1 gm/day/3 days followed by treatment with oral glucocorticoids 0.5-1 mg/kg/day.
- Once remission is achieved, dose is gradually tapered over months. Patients who achieve remission will require long term low dose of corticosteroids.
- Most common side effects:
Cyclophosphamide
- Cyclophosphamide can be admistered in EGPA patients with severe and life threatening multiple organ involvement in addition to glucocorticoids.[7][8]
- Preferred regimen:
- oral therapy: 2 mg/kg/day
- Intravenous pulses: For first 3 doses 15 mg/kg/every 2 weeks, for 4-6 doses 15 mg/kg/ every 3 weeks.
- Common side effects:
- Severe drug induced neutropenia
- Opportunistic infection
- Gonadal toxicity
- Low renal clearance
Maintenance therapy
- Once induction of remission has occured, maintenance therapy with azathioprine or methotrexate can be recommended.[5]
- Maintenance with cyclophosphomide is associated with frequent relapses.
- Preferred regimen: 10-30 mg/day for 12-18 months.
- Folic acid supplementation is necessary during immunosuppressant therapy.
Intravenous immunoglobulins
- High doses of intravenous immunoglobulins may be considered in patients whose flare are refractory to standard therapy and during pregnancy.[9]
- Preferred regimen: 2 g/kg for 2–5-day cycles for every 3-4 weeks.
Plasma exchange
- The benifitial role of plasma exchange in addition to glucocortcoid or immunosuppressieve therapy to improve survival rate in patients with eosinophilic granulomatosis with polyangiitis is unclear.[10]
- Plasma exchange may be considered for patients with severe and rapidly progressieve renal failure, and diffuse alveolar hemorrhage.[11]
References
- ↑ 1.0 1.1 Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L (September 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.
- ↑ Maisch B (February 2015). "[Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine]". Herz (in German). 40 (1): 85–98. doi:10.1007/s00059-014-4200-4. PMID 25676009.
- ↑ Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (January 2011). "The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort". Medicine (Baltimore). 90 (1): 19–27. doi:10.1097/MD.0b013e318205a4c6. PMID 21200183.
- ↑ Yumura W, Kobayashi S, Suka M, Hayashi T, Ito S, Nagafuchi H, Yamada H, Ozaki S (March 2014). "Assessment of the Birmingham vasculitis activity score in patients with MPO-ANCA-associated vasculitis: sub-analysis from a study by the Japanese Study Group for MPO-ANCA-associated vasculitis". Mod Rheumatol. 24 (2): 304–9. doi:10.3109/14397595.2013.854075. PMID 24593206.
- ↑ 5.0 5.1 Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, Puéchal X, Letellier P, Delaval P, Cordier JF, Guillevin L (February 2008). "Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients". Arthritis Rheum. 58 (2): 586–94. doi:10.1002/art.23198. PMID 18240234.
- ↑ Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL (June 2013). "A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients". Ann. Rheum. Dis. 72 (6): 1011–7. doi:10.1136/annrheumdis-2012-201531. PMID 22887848.
- ↑ Guillevin L, Jarrousse B, Lok C, Lhote F, Jais JP, Le Thi Huong Du D, Bussel A (April 1991). "Longterm followup after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomized trial of 71 patients. The Cooperative Study Group for Polyarteritis Nodosa". J. Rheumatol. 18 (4): 567–74. PMID 1676753.
- ↑ Hellmich B, Gross WL (January 2004). "Recent progress in the pharmacotherapy of Churg-Strauss syndrome". Expert Opin Pharmacother. 5 (1): 25–35. doi:10.1517/14656566.5.1.25. PMID 14680433.
- ↑ Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G (December 2004). "Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome". Ann. Rheum. Dis. 63 (12): 1649–54. doi:10.1136/ard.2003.015453. PMC 1754837. PMID 15547090.
- ↑ Guillevin L, Cevallos R, Durand-Gasselin B, Lhote F, Jarrousse B, Callard P (1997). "Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis". Ann Med Interne (Paris). 148 (3): 198–204. PMID 9255326.
- ↑ Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ (December 2003). "Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis". Am. J. Kidney Dis. 42 (6): 1149–53. PMID 14655185.