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. Immunosupressieve agents (eg, azathioprine and methotrexate) can be used for maintenance therapy. Rituximab, interferon-alpha, anti Ig E antibodies and plasma exchange can be used as a second line therapies in the management of eosinophilic granulomatosis with polyangiitis.

Medical Therapy

Initiating pharmacotherapy

Systemic glucocorticosteroids

Cyclophosphamide

Maintenance 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

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

References

  1. 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.
  2. 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.
  3. 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.
  4. 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. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. 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.
  12. 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.
  13. 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.
  14. 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.

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