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Current Rheumatology Reports
Published in: Current Rheumatology Reports 5/2018

01-05-2018 | Vasculitis (L Espinoza, Section Editor)

Eosinophilic Granulomatosis With Polyangiitis: Newer Therapies

Authors: Erika P. Navarro-Mendoza, Gabriel J. Tobón

Published in: Current Rheumatology Reports | Issue 5/2018

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Abstract

Purpose of review

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic disseminated vasculitis associated with extravascular granulomas in patients suffering from asthma and tissue eosinophilia. Current therapies to achieve remission and prevent relapse include glucocorticoids and immunosuppressants like cyclophosphamide.

Recent findings

With the right treatment, clinical prognosis is favorable, so concerted efforts have been made in recent years to find new alternatives for treating severe EGPA. Monoclonal antibodies such as omalizumab, rituximab, and mepolizumab are among these new options.

Summary

This review summarizes the pathogenesis and clinical manifestations of EGPA and critically examines current and emerging therapies.
Literature
1.
•• Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CGM, Lamprecht P. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65:1–11. The current consensus is achieved to describe clinical, radiological, and histological aspects useful in daily practice. PubMed
2.
Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52:2926–35.CrossRefPubMed
3.
Valent P, Klion AD, Horny H, Roufosse F, Gotlib J. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol. 2012;130:607–12.e9.CrossRefPubMedPubMedCentral
4.
Watts RA, Lane S. What is known about the epidemiology of the vasculitides? Best Pract Res Clin Rheumatol. 2005;19:191–207.CrossRefPubMed
5.
Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): state of the art. Allergy. 2013;68:261–73.CrossRefPubMed
6.
Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27:277–301.PubMedPubMedCentral
7.
Somer T, Finefold SM. Vasculitides associated with infections, immunization, and antimicrobial drugs. Clin Infect Dis. 1995;20:1010–36.CrossRefPubMed
8.
Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, et al. HLA–DRB4 as a genetic risk factor for Churg–Strauss syndrome. Arthritis Rheum. 2007;56:3159–66.CrossRefPubMed
9.
Jakiela B, Sanak M, Szczeklik W, Sokolowska B, Plutecka H, Mastalerz L, et al. Both Th2 and Th17 responses are involved in the pathogenesis of Churg–Strauss syndrome. Clin Exp Rheumatol. 2011;29:S23–34.PubMed
10.
Schneeweis C, Rafalowicz M, Feist E, Buttgereit F, Rudolph PE, Burmester GR, et al. Increased levels of BLyS and sVCAM-1 in anti-neutrophil cytoplasmatic antibody (ANCA)-associated vasculitides (AAV). Clin Exp Rheumatol. 2010;28:62–6.PubMed
11.
Mouthon L, Dunoque B, Guillevin L. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome). J Autoimmun. 2014;48-49:99–103.CrossRefPubMed
12.
• Jakiela B, Szczeklik W, Plutecka H, Sokolowska B, Mastalerz L, Sanak M, et al. Increased production of IL-5 and dominant Th2-type response in airways of Churg-Strauss syndrome patients. Rheumatology (Oxford). 2012;51:1887–93. This paper demonstrates the role of Th2 and Il5 in the pathogenesis of EGPA. CrossRef
13.
•• Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–92. Describes the consensus of the Chapel Hill nomenclature for systemic vasculitis. CrossRefPubMed
14.
Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg–Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin N Am. 1995;21:911–47.
15.
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg-Strauss syndrome. Autoimmun Rev. 2015;14:341–8.CrossRefPubMed
16.
Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome. Medicine (Baltimore). 1984;63:65–81.CrossRef
17.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg–Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094–100.CrossRefPubMed
18.
Mukhtyar C, Flossmann O, Hellmich B, Bacon P, Cid M, Cohen-Tervaert JW, et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism Systemic Vasculitis Task Force. Ann Rheum Dis. 2008;67:1004–10.CrossRefPubMed
19.
Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg–Strauss Syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75:17–28.CrossRef
20.
Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015;26:545–53.CrossRefPubMed
21.
Pagnoux C, Guilpain P, Guillevin L. Churg–Strauss syndrome. Curr Opin Rheumatol. 2007;19:25–32.CrossRefPubMed
22.
Samson M, Puéchal X, Devilliers H, Ribi C, Cohen P, Stern M, et al. Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) enrolled in two prospective trials. J Autoimmun. 2013;43:60–9.CrossRefPubMed
23.
De Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis. Ann Intern Med. 2009;150:670–80.CrossRefPubMed
24.
Pagnoux C, Quéméneur T, Ninet J, Perrodeau E, Diot E, Kyndt X, et al. Treatment of systemic necrotizing vasculitides in patients aged sixty-five years or older: results of a multicenter, open-label, randomized controlled trial of corticosteroid and cyclophosphamide-based induction therapy. Arthritis Rheumatol. 2015;67:1117–27.CrossRefPubMed
25.
Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniené J, et al. Randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349:36–44.CrossRefPubMed
26.
Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790–803.CrossRefPubMed
27.
Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771–80.CrossRefPubMed
28.
Puéchal X, Pagnoux C, Baron G, French Vasculitis Study Group, et al. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg-Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors: a randomized, controlled trial. Arthritis Rheumatol. 2017;69:2175–86.CrossRefPubMed
29.
•• Yates M, Watts R, Bajema I, Cid M, Crestani B, Hauser T, et al. Validation of the EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis by disease content experts. RMD Open. 2017;3:e000449. This paper makes the EULAR/ERA-EDTA treatment focus recommendations for EGPA patients based on level evidence. CrossRefPubMedPubMedCentral
30.
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;63:1649–5.CrossRefPubMedPubMedCentral
31.
Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al. Rituximab versus cyclophosphamide in ANCA associated renal vasculitis. N Engl J Med. 2010;363:211–20.CrossRefPubMed
32.
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA associated vasculitis. N Engl J Med. 2010;363:221–32.CrossRefPubMedPubMedCentral
33.
Specks U, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, et al. Efficacy of remission-induction regimens for ANCA associated vasculitis. N Engl J Med. 2013;369:417–27.CrossRefPubMed
34.
Thiel J, Hässler F, Salzer U, Voll RE, Venhoff N. Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg−Strauss syndrome). Arthritis Res Ther. 2013;15:R133.CrossRefPubMedPubMedCentral
35.
Thiel J, Troilo A, Salzer U, Schleyer T, Halmschlag K, Rizzi M. Rituximab as induction therapy in eosinophilic granulomatosis with polyangiitis refractory to conventional immunosuppressive treatment: a 36-month follow-up analysis. J Allergy Clin Immunol Pract. 2017;5:1555–63.CrossRef
36.
Mohamad AJ, Hot A, Arndt F, Moosiq F, Guerry MJ, Amudala N, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Ann Rheum Dis. 2016;75:396–401.CrossRef
37.
Aguirre- Valencia D, Posso-Osorio I, Bravo JC, Bonilla- Abadía F, Tobón GJ, Cañas CA. Sequential rituximab and omalizumab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Clin Rheumatol. 2017;36:2159–62.CrossRefPubMed
38.
Noga O, Hanf G, Brachmann I, Klucken AC, Kleine-Tebbe J, Rosseau S, et al. Effect of omalizumab treatment on peripheral eosinophil and T-lymphocyte function in patients with allergic asthma. J Allergy Clin Immunol. 2006;117:1493–9.CrossRefPubMed
39.
Giavina-Bianchi P, Giavina-Bianchi M, Agondi R, Kalil J. Administration of anti-IgE to a Churg-Strauss syndrome patient. Int Arch Allergy Immunol. 2007;144:155–8.CrossRefPubMed
40.
Detoraki A, Di Capua L, Varricchi G, Genovese A, Marone G, Spadaro G. Omalizumab in patients with eosinophilic granulomatosis with polyangiitis: a 36-month follow-up study. J Asthma. 2016;53:201–6.CrossRefPubMed
41.
• Jachiet M, Samson M, Cottin M, Kahn JE, Le Guenno G, Bonniaud P, et al. Anti-IgE monoclonal antibody (omalizumab) in refractory and relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Arthritis Rheumatol. 2016;68:2274–82. This is the largest study which used omalizumab in EGPA patients. CrossRefPubMed
42.
Nazir S, Tachamo N, Fareedy SB, Khan MS, Lohani S. Omalizumab-associated eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Ann Allergy Asthma Immunol. 2017;118:372–4.e1.CrossRefPubMed
43.
Bekçibaşı M, Barutçu S, Çelen MK, Dayan S, Hoşoğlu S. Churg-Strauss syndrome occurring during omalizumab treatment. Eur J Rheumatol. 2015;2:129–30.CrossRefPubMedPubMedCentral
44.
Cisneros C, Segrelles G, Herráez L, Gonzalez A, Girón R. Churg-Strauss syndrome in a patient treated with omalizumab. J Investig Allergol Clin Immunol. 2013;23:515–6.PubMed
45.
Wechsler ME, Wong DA, Miller MK, Lawrence-Miyasaki L. Churg-Strauss syndrome in patients treated with omalizumab. Chest. 2009;136:507–18.CrossRefPubMed
46.
Puéchal X, Rivereau P, Vinchon F. Churg-Strauss syndrome associated with omalizumab. Eur J Intern Med. 2008;19:364–6.CrossRefPubMed
47.
Gauvreau GM, Boulet LP, Leigh R, Cockcroft DW, Davis BE, Mayers I. QGE031 (ligelizumab) is more effective than omalizumab and placebo in inhibiting allergen-induced early asthmatic response: results from a predictive modeling study. Eur Respir J. 2015;46:PA5091.CrossRef
48.
Bagnasco D, Ferrando M, Varricchhi G, Puggioni F, Passalacqua G, Canonica GW. Anti-interleukin 5 (iL-5) and iL-5Ra biological drugs: efficacy, safety, and future perspectives in severe eosinophilic asthma. Front Med (Lausanne). 2017;4:135.CrossRef
49.
Kahn JE, Grandpeix-Guyodo C, Marroun I, Catherinot E, Mellot F, Roufosse F, et al. Sustained response to mepolizumab in refractory Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:267–70.CrossRefPubMed
50.
Kim S, Marigowda G, Oren E, Israel E, Wechsler ME. Mepolizumab as a steroid sparing treatment option in patients with Churg–Strauss syndrome. J Allergy Clin Immunol. 2010;125:1336–43.CrossRefPubMed
51.
Moosig F, Gross WL, Herrmann K, Bremer JP, Hellmich B. Targeting interleukin-5 in refractory and relapsing Churg–Strauss syndrome. Ann Intern Med. 2011;155:341–3.CrossRefPubMed
52.
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.PubMed
53.
•• Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 2017;376:1921–32. This is the first multicenter double-blind randomized clinical controlled trial that investigates remission in EGPA patient using mepolizumab. CrossRefPubMedPubMedCentral
54.
Guillevin L. Vasculitis: mepolizumab for eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2017;13(9):518–9.CrossRefPubMed
55.
Beck L, Diamant T, Hamilton J, Graham N, Bieber T, Rocklin R, et al. Dupilumab in adults with moderate-to-severe atopic dermatitis. N Engl J Med. 2014;371:130–9.CrossRefPubMed
Metadata
Title
Eosinophilic Granulomatosis With Polyangiitis: Newer Therapies
Authors
Erika P. Navarro-Mendoza
Gabriel J. Tobón
Publication date
01-05-2018
Publisher
Springer US
Published in
Current Rheumatology Reports / Issue 5/2018
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-018-0736-2

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