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
The mainstay of management for eosinophilic granulomatosis with polyangiitis is glucocorticoids and cyclophosphamide. Immunosupressive agents (eg, azathioprine and methotrexate) can be used for maintenance therapy. Rituximab, interferon-alpha, anti IgE antibodies and plasma exchange can be used as a second line therapies in the management of eosinophilic granulomatosis with polyangiitis.
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]
Initiating pharmacotherapy
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:
- Oral dose of 0.5 to 1.0 mg/kg/day for 2-3 weeks then gradual tapering the dose to 0.3 mg/kg/day after 3 months and 0.15 mg/kg/day after 6 months.
- Higher doses of corticosteroids can be given in pulses up to 7.5-15mg/kg/day may be required in severe vasculitis involving heart, kidney, and GIT.
- 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 cyclophosphamide is associated with frequent relapses.
- Preferred regimen: 10-30 mg/day for 12-18 months.
- Folic acid supplementation is necessary during immunosuppressant therapy.
Additional therapy
Rituximab
- Administration of rituximab can be beneficial in ANCA positive patients with progressieve renal failure, CNS involvement, and refractory disease.[9]
Interferon-alpha
- Second line therapy drug, may be useful for patients who are not responding to glucocorticoids or immunosuppressieve agents[10]
- Preferred regimen: Subcutaneous administration of 3 million I.U./3 times weekly
- Relapses are more frequent
Anti-IgE therapy
- Omalizumab may be administered to control asthma, sinusitis and eosinophilia refractory to systemic and inhaled glucocorticoids.[11]
Anti-IL-5 antibodies
- Mepolizumab: Preferred regimen: 300mg given every 4 weeks.
Inhaled glucocorticoids
- They can be used to relieve symptoms of upper airway disease.
Intravenous immunoglobulins
- High doses of intravenous immunoglobulins may be considered in patients whose flare are refractory to standard therapy and during pregnancy.[12]
- Preferred regimen: 2 g/kg for 2–5-day cycles for every 3-4 weeks.
Plasma exchange
- The benificial role of plasma exchange in addition to glucocorticoid or immunosuppressieve therapy to improve survival rate in patients with eosinophilic granulomatosis with polyangiitis is unclear.[13]
- Plasma exchange may be considered for patients with severe and rapidly progressieve renal failure, and diffuse alveolar hemorrhage.[14]
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.
- ↑ Saech J, Owczarczyk K, Owczarzyk K, Rösgen S, Petereit H, Hallek M, Rubbert-Roth A (June 2010). "Successful use of rituximab in a patient with Churg-Strauss syndrome and refractory central nervous system involvement". Ann. Rheum. Dis. 69 (6): 1254–5. doi:10.1136/ard.2009.109850. PMID 19740901.
- ↑ Metzler C, Schnabel A, Gross WL, Hellmich B (2008). "A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome". Clin. Exp. Rheumatol. 26 (3 Suppl 49): S35–40. PMID 18799051.
- ↑ Jachiet M, Samson M, Cottin V, Kahn JE, Le Guenno G, Bonniaud P, Devilliers H, Bouillet L, Gondouin A, Makhlouf F, Meaux-Ruault N, Gil H, Bienvenu B, Coste A, Groh M, Giraud V, Dominique S, Godeau B, Puéchal X, Khouatra C, Ruivard M, Le Jeunne C, Mouthon L, Guillevin L, Terrier B (September 2016). "Anti-IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss): Data on Seventeen Patients". Arthritis Rheumatol. 68 (9): 2274–82. doi:10.1002/art.39663. PMID 26946346.
- ↑ 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.