Successful treatment of eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg–Strauss syndrome) with rituximab in a case refractory to glucocorticoids, cyclophosphamide, and IVIG

  • Natsuka Umezawa
  • Hitoshi Kohsaka
  • Toshihiro Nanki
  • Kaori Watanabe
  • Michi Tanaka
  • Peter Y. Shane
  • Nobuyuki Miyasaka
Case Report

Abstract

A 44-year old woman with eosinophilic granulomatosis with polyangiitis (EGPA) developed sequential paralysis of different cranial nerves despite treatments including methylpredonisolone pulse therapy, intravenous immunoglobulins (IVIG), and cyclophosphamide. Infusions of rituximab ameliorated her neurological symptoms and serological inflammatory findings. Rituximab, a specific B cell-targeting therapy, might offer an alternative for refractory EGPA with possible advantages of cost and ease of use compared to IVIG, which also targets (at least in part) B lymphocytes and immunoglobulin production.

Keywords

Eosinophilic granulomatosis with polyangiitis IVIG Rituximab 

References

  1. 1.
    Cohen P, Pagnoux C, Mahr A, Arene JP, Mouthon L, Le Guern V, et al. Churg–Strauss syndrome with poor-prognosis factors: a prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients. Arthritis Rheum. 2007;57:686–93.Google Scholar
  2. 2.
    Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68:310–17.Google Scholar
  3. 3.
    Taniguchi M, Tsurikisawa N, Higashi N, Saito H, Mita H, Mori A, et al. Treatment for Churg–Strauss syndrome: induction of remission and efficacy of intravenous immunoglobulin therapy. Allergol Int. 2007;56:97–103.PubMedCrossRefGoogle Scholar
  4. 4.
    Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. J N Eng Med. 2010;363:211–20.CrossRefGoogle Scholar
  5. 5.
    Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Eng J Med. 2010;363:221–32.CrossRefGoogle Scholar
  6. 6.
    Kaushik VV, Reddy HV, Bucknall RC. Successful use of rituximab in a patient with recalcitrant Churg–Strauss syndrome. Ann Rheum Dis. 2006;65:1116–7.PubMedCrossRefGoogle Scholar
  7. 7.
    Koukoulaki M, Smith KG, Jayne DR. Rituximab in Churg–Strauss syndrome. Ann Rheum Dis. 2006;65:557–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Pepper RJ, Fabre MA, Pavesio C, Gaskin G, Jones RB, Jayne D, et al. Rituximab is effective in the treatment of refractory Churg–Strauss syndrome and is associated with diminished T-cell interleukin-5 production. Rheumatology (Oxford). 2008;47:1104–5.CrossRefGoogle Scholar
  9. 9.
    Saech J, Owczarczyk K, Rosgen S, Petereit H, Hallek M, Rubbert-Roth A. Successful use of rituximab in a patient with Churg–Strauss syndrome and refractory central nervous system involvement. Ann Rheum Dis. 2010;69:1254–5.PubMedCrossRefGoogle Scholar
  10. 10.
    Donvik KK, Omdal R. Churg–Strauss syndrome successfully treated with rituximab. Rheumatol Int. 2011;31:89–91.PubMedCrossRefGoogle Scholar
  11. 11.
    Cartin-Ceba R, Keogh KA, Specks U, Sethi S, Fervenza FC. Rituximab for the treatment of Churg–Strauss syndrome with renal involvement. Nephrol Dial Transplant. 2011;26:2865–71.PubMedCrossRefGoogle Scholar
  12. 12.
    Sewell WA, Jolles S. Immunomodulatory action of intravenous immunoglobulin. Immunology. 2002;107:387–93.PubMedCrossRefGoogle Scholar
  13. 13.
    Tsurikisawa N, Saito H, Oshikata C, Tsuburai T, Akiyama K. High-dose intravenous immunoglobulin treatment increases regulatory T cells in patients with eosinophilic granulomatosis with polyangiitis. J Rheumatol. 2012;39:1019–25.PubMedCrossRefGoogle Scholar
  14. 14.
    Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G. Long term effectiveness of intravenous immunoglobulin in Churg–Strauss syndrome. Ann Rheum Dis. 2004;63:1649–54.PubMedCrossRefGoogle Scholar
  15. 15.
    Herrmann K, Gross WL, Moosig F. Extended follow-up after stopping mepolizumab in relapsing/refractory Churg–Strauss syndrome. Clin Exp Rheumatol. 2012;30:S62–5.PubMedGoogle Scholar
  16. 16.
    Guilpain P, Auclair JF, Tamby MC, Servettaz A, Mahr A, Weill B, et al. Serum eosinophil cationic protein: a marker of disease activity in Churg–Strauss syndrome. Ann N Y Acad Sci. 2007;1107:392–9.PubMedCrossRefGoogle Scholar
  17. 17.
    Hurst S, Chizzolini C, Dayer JM, Olivieri J, Roux-Lombard P. Usefulness of serum eosinophil cationic protein (ECP) in predicting relapse of Churg and Strauss vasculitis. Clin Exp Rheumatol. 2000;18:784–5.PubMedGoogle Scholar

Copyright information

© Japan College of Rheumatology 2012

Authors and Affiliations

  • Natsuka Umezawa
    • 1
  • Hitoshi Kohsaka
    • 1
  • Toshihiro Nanki
    • 1
    • 2
  • Kaori Watanabe
    • 1
    • 2
  • Michi Tanaka
    • 1
    • 2
  • Peter Y. Shane
    • 1
  • Nobuyuki Miyasaka
    • 1
  1. 1.Department of Medicine and Rheumatology, Graduate School of Medicine and Dental ScienceTokyo Medical and Dental UniversityTokyoJapan
  2. 2.Department of PharmacovigilanceTokyo Medical and Dental UniversityTokyoJapan

Personalised recommendations