Skip to main content
Top
Published in: Allergy, Asthma & Clinical Immunology 1/2023

Open Access 01-12-2023 | Vasculitis | Research

Long-term mepolizumab treatment reduces relapse rates in super-responders with eosinophilic granulomatosis with polyangiitis

Authors: Nami Masumoto, Chiyako Oshikata, Ryo Nakadegawa, Yuto Motobayashi, Reeko Osada, Saki Manabe, Takeshi Kaneko, Naomi Tsurikisawa

Published in: Allergy, Asthma & Clinical Immunology | Issue 1/2023

Login to get access

Abstract

Background

The mainstay of treatment for eosinophilic granulomatosis with polyangiitis (EGPA) is systemic corticosteroid therapy; some patients also receive intravenous immunoglobulins, other immunosuppressive agents, and biologics. Mepolizumab, an anti-interleukin-5 monoclonal antibody, induces remission and decreases the daily corticosteroid dose; however, the clinical efficacy of mepolizumab in EGPA and the prognosis with long-term treatment with this drug are unknown.

Methods

Seventy-one EGPA patients were treated at Hiratsuka City Hospital, Japan, between April 2018 and March 2022. We administered mepolizumab for a mean of 2.8 ± 1.7 years to 43 patients in whom remission could not be induced by conventional treatment. After excluding 18 patients who had received mepolizumab for less than 3 years, we classified 15 patients into a “super-responder group” (the daily dose of corticosteroids or other immunosuppressant could be decreased, or the interval between IVIG treatments could be prolonged) and 10 patients into a “responder group” (neither of these changes was achievable). Eosinophil numbers, serum IgG levels, daily doses of corticosteroids and other immunosuppressants, Birmingham Vasculitis Activity Score (BVAS), and relapse frequency before and after mepolizumab initiation were determined.

Results

Blood eosinophil count at diagnosis and the lowest serum IgG level before mepolizumab treatment were significantly higher in super-responders than in responders (p < 0.05). In super-responders, the prednisolone dose at last visit on mepolizumab treatment was lower than that before treatment (p < 0.01) and lower than that at last visit in the responders (p < 0.01). In both groups, peripheral blood eosinophil numbers and BVAS were lower after starting mepolizumab than before (p < 0.01). BVAS before mepolizumab (p < 0.05) and at last visit (p < 0.01) were lower in super-responders than in responders. Relapse rates every year after the start of mepolizumab were lower in super-responders than in responder groups (p < 0.01). In super-responders, relapse rates were lower during the 3 years following mepolizumab initiation (p < 0.01) and at last visit (p < 0.01) were significantly lower than after 1 year of treatment.

Conclusion

Mepolizumab treatment of super-responders sustainably reduced the relapse rate.
Literature
1.
go back to reference Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27:277–301.PubMedPubMedCentral Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27:277–301.PubMedPubMedCentral
2.
go back to reference Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013;17:603–6.PubMedPubMedCentralCrossRef Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013;17:603–6.PubMedPubMedCentralCrossRef
3.
go back to reference Churg A. Recent advances in the diagnosis of Churg-Strauss syndrome. Mod Pathol. 2001;14:1284–93.PubMedCrossRef Churg A. Recent advances in the diagnosis of Churg-Strauss syndrome. Mod Pathol. 2001;14:1284–93.PubMedCrossRef
5.
6.
go back to reference Terrier B, Bièche I, Maisonobe T, Laurendeau I, Rosenzwajg M, Kahn JE, et al. Interleukin-25: a cytokine linking eosinophils and adaptive immunity in Churg-Strauss syndrome. Blood. 2010;116:4523–31.PubMedCrossRef Terrier B, Bièche I, Maisonobe T, Laurendeau I, Rosenzwajg M, Kahn JE, et al. Interleukin-25: a cytokine linking eosinophils and adaptive immunity in Churg-Strauss syndrome. Blood. 2010;116:4523–31.PubMedCrossRef
7.
go back to reference 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. 2012;51:1887–93.PubMedCrossRef 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. 2012;51:1887–93.PubMedCrossRef
8.
go back to reference 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(1 Suppl 64):S23-34.PubMed 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(1 Suppl 64):S23-34.PubMed
9.
go back to reference Tsurikisawa N, Oshikata C, Watanabe M, Tsuburai T, Kaneko T, Saito H. Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis. Clin Exp Allergy. 2018;48:1305–16.PubMedCrossRef Tsurikisawa N, Oshikata C, Watanabe M, Tsuburai T, Kaneko T, Saito H. Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis. Clin Exp Allergy. 2018;48:1305–16.PubMedCrossRef
10.
go back to reference Abril A, Calamia KT, Cohen MD. The Churg-Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum. 2003;33:106–14.PubMedCrossRef Abril A, Calamia KT, Cohen MD. The Churg-Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum. 2003;33:106–14.PubMedCrossRef
11.
go back to reference SeeligerFörsterHappeForbergMoeserNeumann BMJTAT, et al. Interferon-α for induction and maintenance of remission in eosinophilic granulomatosis with polyangiitis: a single-center retrospective observational cohort study. Rheumatol. 2017;44:806–14. SeeligerFörsterHappeForbergMoeserNeumann BMJTAT, et al. Interferon-α for induction and maintenance of remission in eosinophilic granulomatosis with polyangiitis: a single-center retrospective observational cohort study. Rheumatol. 2017;44:806–14.
12.
go back to reference 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.PubMedCrossRef 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.PubMedCrossRef
13.
go back to reference Fanouriakis A, Kougkas N, Vassilopoulos D, Fragouli E, Repa A, Sidiropoulos P. Rituximab for eosinophilic granulomatosis with polyangiitis with severe vasculitic neuropathy: case report and review of current clinical evidence. Semin Arthritis Rheum. 2015;45:60–6.PubMedCrossRef Fanouriakis A, Kougkas N, Vassilopoulos D, Fragouli E, Repa A, Sidiropoulos P. Rituximab for eosinophilic granulomatosis with polyangiitis with severe vasculitic neuropathy: case report and review of current clinical evidence. Semin Arthritis Rheum. 2015;45:60–6.PubMedCrossRef
14.
go back to reference Mohammad AJ, Hot A, Arndt F, Moosig 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.PubMedCrossRef Mohammad AJ, Hot A, Arndt F, Moosig 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.PubMedCrossRef
15.
go back to reference Tsurikisawa N, Oshikata C, Kinoshita A, Tsuburai T, Saito H. Long-term prognosis of 121 patients with eosinophilic granulomatosis with polyangiitis in Japan. J Rheumatol. 2017;44:1206–15.PubMedCrossRef Tsurikisawa N, Oshikata C, Kinoshita A, Tsuburai T, Saito H. Long-term prognosis of 121 patients with eosinophilic granulomatosis with polyangiitis in Japan. J Rheumatol. 2017;44:1206–15.PubMedCrossRef
16.
go back to reference Martinez V, Cohen P, Pagnoux C, Vinzio S, Mahr A, Mouthon L, et al. Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum. 2008;58:308–17.PubMedCrossRef Martinez V, Cohen P, Pagnoux C, Vinzio S, Mahr A, Mouthon L, et al. Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum. 2008;58:308–17.PubMedCrossRef
17.
go back to reference Tsurikisawa N, Taniguchi T, Saito H, Himeno H, Ishibashi A, Suzuki S, et al. Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin. Ann Allergy Asthma Immunol. 2004;92:80–7.PubMedCrossRef Tsurikisawa N, Taniguchi T, Saito H, Himeno H, Ishibashi A, Suzuki S, et al. Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin. Ann Allergy Asthma Immunol. 2004;92:80–7.PubMedCrossRef
18.
go back to reference Ford JA, Aleatany Y, Gewurz-Singer O. Therapeutic advances in eosinophilic granulomatosis with polyangiitis. Curr Opin Rheumatol. 2022;34:158–64.PubMedCrossRef Ford JA, Aleatany Y, Gewurz-Singer O. Therapeutic advances in eosinophilic granulomatosis with polyangiitis. Curr Opin Rheumatol. 2022;34:158–64.PubMedCrossRef
20.
go back to reference Basta F, Mazzuca C, Nucera E, Schiavino D, Afeltra A, Antonelli Incalzi R. Omalizumab in eosinophilic granulomatosis with polyangiitis: friend or foe? A systematic literature review. Clin Exp Rheumatol. 2020;124(2):214–20. Basta F, Mazzuca C, Nucera E, Schiavino D, Afeltra A, Antonelli Incalzi R. Omalizumab in eosinophilic granulomatosis with polyangiitis: friend or foe? A systematic literature review. Clin Exp Rheumatol. 2020;124(2):214–20.
21.
go back to reference Canzian A, Venhoff N, Urban ML, et al. Use of Biologics to treat relapsing and/or refractory eosinophilic granulomatosis with polyangiitis: data from a European collaborative study. Arthritis Rheumatol. 2021;73:498–503.PubMedCrossRef Canzian A, Venhoff N, Urban ML, et al. Use of Biologics to treat relapsing and/or refractory eosinophilic granulomatosis with polyangiitis: data from a European collaborative study. Arthritis Rheumatol. 2021;73:498–503.PubMedCrossRef
22.
go back to reference Kim S, Marigowda G, Oren E, Israel E, Wechsler ME. Mepolizumab as a steroidsparing treatment option in patients with Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:1336–43.PubMedCrossRef Kim S, Marigowda G, Oren E, Israel E, Wechsler ME. Mepolizumab as a steroidsparing treatment option in patients with Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:1336–43.PubMedCrossRef
23.
go back to reference Kahn JE, Grandpeix-Guyodo C, Marroun I, et al. Sustained response to mepolizumab in refractory Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:267–70.PubMedCrossRef Kahn JE, Grandpeix-Guyodo C, Marroun I, et al. Sustained response to mepolizumab in refractory Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:267–70.PubMedCrossRef
24.
go back to reference 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.PubMedCrossRef 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.PubMedCrossRef
25.
go back to reference 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.PubMedPubMedCentralCrossRef 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.PubMedPubMedCentralCrossRef
26.
go back to reference Pavord ID, Korn S, Howarth P, Bleecker ER, Buhl R, Keene ON, et al. Mepolizumab for severe eosinophilic asthma (DREAM): a multicentre, double-blind, placebo-controlled trial. Lancet. 2012;380:651–9.PubMedCrossRef Pavord ID, Korn S, Howarth P, Bleecker ER, Buhl R, Keene ON, et al. Mepolizumab for severe eosinophilic asthma (DREAM): a multicentre, double-blind, placebo-controlled trial. Lancet. 2012;380:651–9.PubMedCrossRef
27.
go back to reference Ortega HG, Liu MC, Pavord ID, Brusselle GG, FitzGerald JM, Chetta A, et al. Mepolizumab treatment in patients with severe eosinophilic asthma. N Engl J Med. 2014;371:1198–207.PubMedCrossRef Ortega HG, Liu MC, Pavord ID, Brusselle GG, FitzGerald JM, Chetta A, et al. Mepolizumab treatment in patients with severe eosinophilic asthma. N Engl J Med. 2014;371:1198–207.PubMedCrossRef
28.
go back to reference Tsurikisawa N, Oshikata C, Watanabe M, Fukuda N, Yamaguchi T, Kiyohara H, Kaneko T. Clinical features of patients with active eosinophilic granulomatosis with polyangiitis successfully treated with mepolizumab. Int Arch Allergy Immunol. 2021;182:744–56.PubMedCrossRef Tsurikisawa N, Oshikata C, Watanabe M, Fukuda N, Yamaguchi T, Kiyohara H, Kaneko T. Clinical features of patients with active eosinophilic granulomatosis with polyangiitis successfully treated with mepolizumab. Int Arch Allergy Immunol. 2021;182:744–56.PubMedCrossRef
29.
go back to reference Koga Y, Aoki-Saito H, Kamide Y, Sato M, Tsurumaki H, Yatomi M, Ishizuka T, Hisada T. Perspectives on the efficacy of benralizumab for treatment of eosinophilic granulomatosis with polyangiitis. Front Pharmacol. 2022;13: 865318.PubMedPubMedCentralCrossRef Koga Y, Aoki-Saito H, Kamide Y, Sato M, Tsurumaki H, Yatomi M, Ishizuka T, Hisada T. Perspectives on the efficacy of benralizumab for treatment of eosinophilic granulomatosis with polyangiitis. Front Pharmacol. 2022;13: 865318.PubMedPubMedCentralCrossRef
31.
go back to reference Navarro-Mendoza EP, Tobón GJ. Eosinophilic granulomatosis with polyangiitis: newer therapies. Curr Rheumatol Rep. 2018;20:23.PubMedCrossRef Navarro-Mendoza EP, Tobón GJ. Eosinophilic granulomatosis with polyangiitis: newer therapies. Curr Rheumatol Rep. 2018;20:23.PubMedCrossRef
32.
go back to reference Ueno M, Miyagawa I, Nakano K, Iwata S, Hanami K, Fukuyo S, Kubo S, Miyazaki Y, Kawabe A, Yoshinari H, Nakayamada S, Tanaka Y. Effectiveness and safety of mepolizumab in combination with corticosteroids in patients with eosinophilic granulomatosis with polyangiitis. Arthritis Res Ther. 2021;23:86.PubMedPubMedCentralCrossRef Ueno M, Miyagawa I, Nakano K, Iwata S, Hanami K, Fukuyo S, Kubo S, Miyazaki Y, Kawabe A, Yoshinari H, Nakayamada S, Tanaka Y. Effectiveness and safety of mepolizumab in combination with corticosteroids in patients with eosinophilic granulomatosis with polyangiitis. Arthritis Res Ther. 2021;23:86.PubMedPubMedCentralCrossRef
33.
go back to reference 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.PubMedCrossRef 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.PubMedCrossRef
34.
go back to reference Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. French Vasculitis Study Group. eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65:270–81.PubMedCrossRef Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. French Vasculitis Study Group. eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65:270–81.PubMedCrossRef
35.
go back to reference Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin P. The five-factor score revisited. assessment of prognosis of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) Cohort. Medicine. 2011;90:19–27.PubMedCrossRef Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin P. The five-factor score revisited. assessment of prognosis of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) Cohort. Medicine. 2011;90:19–27.PubMedCrossRef
36.
go back to reference Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, et al. 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. 2013;72:1011–7.PubMedCrossRef Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, et al. 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. 2013;72:1011–7.PubMedCrossRef
37.
go back to reference Hanania NA, Niven R, Chanez P, Antoine D, Pfister P, Garcia Conde L, Jaumont X. Long-term effectiveness and safety of omalizumab in pediatric and adult patients with moderate-to-severe inadequately controlled allergic asthma. World Allergy Organ J. 2022;15: 100695.PubMedPubMedCentralCrossRef Hanania NA, Niven R, Chanez P, Antoine D, Pfister P, Garcia Conde L, Jaumont X. Long-term effectiveness and safety of omalizumab in pediatric and adult patients with moderate-to-severe inadequately controlled allergic asthma. World Allergy Organ J. 2022;15: 100695.PubMedPubMedCentralCrossRef
38.
go back to reference Eger K, Kroes JA, Ten Brinke A, Bel EH. Long-term therapy response to anti-IL-5 biologics in severe asthma-a real-life evaluation. J Allergy Clin Immunol Pract. 2021;9:1194–200.PubMedCrossRef Eger K, Kroes JA, Ten Brinke A, Bel EH. Long-term therapy response to anti-IL-5 biologics in severe asthma-a real-life evaluation. J Allergy Clin Immunol Pract. 2021;9:1194–200.PubMedCrossRef
39.
go back to reference Murphy K, Jacobs J, Bjermer L, Fahrenholz JM, Shalit Y, Garin M, Zangrilli J, Castro M. Long-term safety and efficacy of reslizumab in patients with eosinophilic asthma. J Allergy Clin Immunol Pract. 2017;5:1572-81.e3.PubMedCrossRef Murphy K, Jacobs J, Bjermer L, Fahrenholz JM, Shalit Y, Garin M, Zangrilli J, Castro M. Long-term safety and efficacy of reslizumab in patients with eosinophilic asthma. J Allergy Clin Immunol Pract. 2017;5:1572-81.e3.PubMedCrossRef
40.
go back to reference Sposato B, Scalese M, Camiciottoli G, Carpagnano GE, Pelaia C, Santus P, Pelaia G, Palmiero G, Di Tomassi M, Ronchi MC, Cameli P, Bargagli E, Ciambellotti L, Rizzello S, Sglavo R, Coppola A, Lacerenza LG, Gabriele M, Radovanovic D, Perrella A, Ricci A, Rogliani P. Severe asthma and long-term Benralizumab effectiveness in real-life. Eur Rev Med Pharmacol Sci. 2022;26:7461–73.PubMed Sposato B, Scalese M, Camiciottoli G, Carpagnano GE, Pelaia C, Santus P, Pelaia G, Palmiero G, Di Tomassi M, Ronchi MC, Cameli P, Bargagli E, Ciambellotti L, Rizzello S, Sglavo R, Coppola A, Lacerenza LG, Gabriele M, Radovanovic D, Perrella A, Ricci A, Rogliani P. Severe asthma and long-term Benralizumab effectiveness in real-life. Eur Rev Med Pharmacol Sci. 2022;26:7461–73.PubMed
41.
go back to reference Ozaki S. ANCA-associated vasculitis: diagnostic and therapeutic strategy. Allergol Int. 2007;56:87–96.PubMedCrossRef Ozaki S. ANCA-associated vasculitis: diagnostic and therapeutic strategy. Allergol Int. 2007;56:87–96.PubMedCrossRef
42.
go back to reference Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine. 1999;78:26–37.PubMedCrossRef Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine. 1999;78:26–37.PubMedCrossRef
43.
go back to reference Silva CI, Müller NL, Fujimoto K, Johkoh T, Ajzen SA, Churg A. Churg-Strauss syndrome: high resolution CT and pathologic findings. J Thorac Imaging. 2005;20:74–80.PubMedCrossRef Silva CI, Müller NL, Fujimoto K, Johkoh T, Ajzen SA, Churg A. Churg-Strauss syndrome: high resolution CT and pathologic findings. J Thorac Imaging. 2005;20:74–80.PubMedCrossRef
44.
go back to reference Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review Am J Clin Pathol. 2000;114:767–72.PubMedCrossRef Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review Am J Clin Pathol. 2000;114:767–72.PubMedCrossRef
45.
go back to reference Lesens O, Hansmann Y, Nerson J, Pasquali J, Gasser B, Wihlm J, et al. Severe Churg-Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy. Eur J Intern Med. 2002;13:458.PubMedCrossRef Lesens O, Hansmann Y, Nerson J, Pasquali J, Gasser B, Wihlm J, et al. Severe Churg-Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy. Eur J Intern Med. 2002;13:458.PubMedCrossRef
46.
go back to reference Choi JY, Kim JE, Choi IY, Lee JH, Kim JH, Shin C, et al. Churg-Strauss syndrome that presented with mediastinal lymphadenopathy and calculous cholecystitis. Korean J Intern Med. 2016;31:179–83.PubMedCrossRef Choi JY, Kim JE, Choi IY, Lee JH, Kim JH, Shin C, et al. Churg-Strauss syndrome that presented with mediastinal lymphadenopathy and calculous cholecystitis. Korean J Intern Med. 2016;31:179–83.PubMedCrossRef
47.
go back to reference Worthy SA, Müller NL, Hansell DM, Flower CD. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. Am J Roentgenol. 1998;170:297–300.CrossRef Worthy SA, Müller NL, Hansell DM, Flower CD. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. Am J Roentgenol. 1998;170:297–300.CrossRef
48.
go back to reference Tsurikisawa N, Oshikata C, Tsuburai T, Sugano S, Nakamura Y, Shimoda T, et al. Th17 cells reflect colon submucosal pathologic changes in active eosinophilic granulomatosis with polyangiitis. BMC Immunol. 2015;16:75.PubMedPubMedCentralCrossRef Tsurikisawa N, Oshikata C, Tsuburai T, Sugano S, Nakamura Y, Shimoda T, et al. Th17 cells reflect colon submucosal pathologic changes in active eosinophilic granulomatosis with polyangiitis. BMC Immunol. 2015;16:75.PubMedPubMedCentralCrossRef
49.
go back to reference Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. Birmingham vasculitis activity score (BVAS) in systemic necrotizing vasculitis. Q J Med. 1994;87:671–8. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. Birmingham vasculitis activity score (BVAS) in systemic necrotizing vasculitis. Q J Med. 1994;87:671–8.
50.
go back to reference Molfino NA, Gossage D, Kolbeck R, Parker JM, Geba GP. Molecular and clinical rationale for therapeutic targeting of interleukin-5 and its receptor. Clin Exp Allergy. 2012;42:712–37.PubMedCrossRef Molfino NA, Gossage D, Kolbeck R, Parker JM, Geba GP. Molecular and clinical rationale for therapeutic targeting of interleukin-5 and its receptor. Clin Exp Allergy. 2012;42:712–37.PubMedCrossRef
51.
go back to reference Zia-Amirhosseini P, Minthorn E, Benincosa LJ, Hart TK, Hottenstein CS, Tobia LA, et al. Pharmacokinetics and pharmacodynamics of SB-240563, a humanized monoclonal antibody directed to human interleukin-5, in monkeys. J Pharmacol Exp Ther. 1999;291:1060–7.PubMed Zia-Amirhosseini P, Minthorn E, Benincosa LJ, Hart TK, Hottenstein CS, Tobia LA, et al. Pharmacokinetics and pharmacodynamics of SB-240563, a humanized monoclonal antibody directed to human interleukin-5, in monkeys. J Pharmacol Exp Ther. 1999;291:1060–7.PubMed
52.
go back to reference Leckie MJ, At B, Khan J, Diamant Z, O’Connor BJ, Walls CM, et al. Effects of an interleukin-5 blocking monoclonal antibody on eosinophils, airway hyper-responsiveness, and the late asthmatic response. Lancet. 2000;356(9248):2144–8.PubMedCrossRef Leckie MJ, At B, Khan J, Diamant Z, O’Connor BJ, Walls CM, et al. Effects of an interleukin-5 blocking monoclonal antibody on eosinophils, airway hyper-responsiveness, and the late asthmatic response. Lancet. 2000;356(9248):2144–8.PubMedCrossRef
53.
go back to reference Nara M, Saito M, Abe F, Komatsuda A, Wakui H, Takahashi N. A pediatric case of relapsing eosinophilic granulomatosis with polyangiitis successfully treated with mepolizumab. Intern Med. 2019;58:3583–7.PubMedPubMedCentralCrossRef Nara M, Saito M, Abe F, Komatsuda A, Wakui H, Takahashi N. A pediatric case of relapsing eosinophilic granulomatosis with polyangiitis successfully treated with mepolizumab. Intern Med. 2019;58:3583–7.PubMedPubMedCentralCrossRef
55.
go back to reference Ueki Y, Oshikata C, Asai Y, Kaneko T, Tsurikisawa N. Familial eosinophilic granulomatosis with polyangiitis in sister and brother. Intern Med. 2020;59:991–5.PubMedCrossRef Ueki Y, Oshikata C, Asai Y, Kaneko T, Tsurikisawa N. Familial eosinophilic granulomatosis with polyangiitis in sister and brother. Intern Med. 2020;59:991–5.PubMedCrossRef
56.
go back to reference Kasuya A, Kitano S, Hoshino T, Ishibe J, Imura K, Goto H, et al. Successful control of severe eosinophilic granulomatosis with polyangiitis in pregnancy and perinatal period: a use of mepolizumab. J Dermatol. 2019;46:e309–11.PubMedCrossRef Kasuya A, Kitano S, Hoshino T, Ishibe J, Imura K, Goto H, et al. Successful control of severe eosinophilic granulomatosis with polyangiitis in pregnancy and perinatal period: a use of mepolizumab. J Dermatol. 2019;46:e309–11.PubMedCrossRef
57.
go back to reference Guillevin L. Vasculitis: mepolizumab for eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2017;13:518–9.PubMedCrossRef Guillevin L. Vasculitis: mepolizumab for eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2017;13:518–9.PubMedCrossRef
58.
go back to reference Ennis D, Lee JK, Pagnoux C. Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis. Expert Opin Biol Ther. 2019;19:617–30.PubMedCrossRef Ennis D, Lee JK, Pagnoux C. Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis. Expert Opin Biol Ther. 2019;19:617–30.PubMedCrossRef
59.
go back to reference Tsurikisawa N, Saito H, Oshikata C, Tsuburai T, Akiyama K. Decreases in the numbers of peripheral blood regulatory T cells, and increases in the levels of memory and activated B cells, in patients with active eosinophilic granulomatosis and polyangiitis. J Clin Immunol. 2013;33:965–76.PubMedCrossRef Tsurikisawa N, Saito H, Oshikata C, Tsuburai T, Akiyama K. Decreases in the numbers of peripheral blood regulatory T cells, and increases in the levels of memory and activated B cells, in patients with active eosinophilic granulomatosis and polyangiitis. J Clin Immunol. 2013;33:965–76.PubMedCrossRef
60.
go back to reference Ríos-Garcés R, Prieto-González S, Hernández-Rodríguez J, et al. Response to mepolizumab according to disease manifestations in patients with eosinophilic granulomatosis with polyangiitis. Eur J Intern Med. 2022;95:61–6.PubMedCrossRef Ríos-Garcés R, Prieto-González S, Hernández-Rodríguez J, et al. Response to mepolizumab according to disease manifestations in patients with eosinophilic granulomatosis with polyangiitis. Eur J Intern Med. 2022;95:61–6.PubMedCrossRef
61.
go back to reference Tsurikisawa N, Saito H, Tsuburai T, Oshikata C, Ono E, Mitomi H, Akiyama K. Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma. J Allergy Clin Immunol. 2008;122:610–6.PubMedCrossRef Tsurikisawa N, Saito H, Tsuburai T, Oshikata C, Ono E, Mitomi H, Akiyama K. Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma. J Allergy Clin Immunol. 2008;122:610–6.PubMedCrossRef
62.
go back to reference Tsurikisawa N, Saito H, Oshikata C, Tsuburai T, Akiyama K. High-dose intravenous immunoglobulin therapy for eosinophilic granulomatosis with polyangiitis. Clinical and Translational Allergy. 2014;4:38.PubMedPubMedCentralCrossRef Tsurikisawa N, Saito H, Oshikata C, Tsuburai T, Akiyama K. High-dose intravenous immunoglobulin therapy for eosinophilic granulomatosis with polyangiitis. Clinical and Translational Allergy. 2014;4:38.PubMedPubMedCentralCrossRef
63.
go back to reference 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.PubMedCrossRef 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.PubMedCrossRef
64.
go back to reference Matsuyama T, Matsuyama H, Dotake Y, Takagi K, Machida K, Inoue H. The therapeutic potential for targeting group 2 innate lymphoid cells in asthma. Front Immunol. 2022;13: 930862.PubMedPubMedCentralCrossRef Matsuyama T, Matsuyama H, Dotake Y, Takagi K, Machida K, Inoue H. The therapeutic potential for targeting group 2 innate lymphoid cells in asthma. Front Immunol. 2022;13: 930862.PubMedPubMedCentralCrossRef
65.
go back to reference Kotas ME, Dion J, Van Dyken S, Ricardo-Gonzalez RR, Danel CJ, Taillé C, Mouthon L, Locksley RM, Terrier B. A role for IL-33-activated ILC2s in eosinophilic vasculitis. JCI Insight. 2021;6: e143366.PubMedPubMedCentralCrossRef Kotas ME, Dion J, Van Dyken S, Ricardo-Gonzalez RR, Danel CJ, Taillé C, Mouthon L, Locksley RM, Terrier B. A role for IL-33-activated ILC2s in eosinophilic vasculitis. JCI Insight. 2021;6: e143366.PubMedPubMedCentralCrossRef
66.
go back to reference Sakthivel P, Bruder D. Mechanism of granuloma formation in sarcoidosis. Curr Opin Hematol. 2017;24:59–65.PubMedCrossRef Sakthivel P, Bruder D. Mechanism of granuloma formation in sarcoidosis. Curr Opin Hematol. 2017;24:59–65.PubMedCrossRef
67.
go back to reference Cosmi L. Th17 and Treg lymphocytes as cellular biomarkers of disease activity in granulomatosis with polyangiitis. Eur J Immunol. 2017;47(4):633–6.PubMedCrossRef Cosmi L. Th17 and Treg lymphocytes as cellular biomarkers of disease activity in granulomatosis with polyangiitis. Eur J Immunol. 2017;47(4):633–6.PubMedCrossRef
68.
go back to reference Tsukamoto N, Takahashi N, Itoh H, Pouliquen I. Pharmacokinetics and pharmacodynamics of mepolizumab, an anti-interleukin 5 monoclonal antibody, in healthy Japanese male subjects. Clin Pharmacol Drug Dev. 2016;5:102–8.PubMedCrossRef Tsukamoto N, Takahashi N, Itoh H, Pouliquen I. Pharmacokinetics and pharmacodynamics of mepolizumab, an anti-interleukin 5 monoclonal antibody, in healthy Japanese male subjects. Clin Pharmacol Drug Dev. 2016;5:102–8.PubMedCrossRef
69.
go back to reference Kavanagh JE, d’Ancona G, Elstad M, Green L, Fernandes M, Thomson L, Roxas C, Dhariwal J, Nanzer AM, Kent BD, Jackson DJ. Real-world effectiveness and the characteristics of a “super-responder” to mepolizumab in severe eosinophilic asthma. Chest. 2020;158:491–500.PubMedCrossRef Kavanagh JE, d’Ancona G, Elstad M, Green L, Fernandes M, Thomson L, Roxas C, Dhariwal J, Nanzer AM, Kent BD, Jackson DJ. Real-world effectiveness and the characteristics of a “super-responder” to mepolizumab in severe eosinophilic asthma. Chest. 2020;158:491–500.PubMedCrossRef
70.
go back to reference Loureiro CC, Amaral L, Ferreira JA, Lima R, Pardal C, Fernandes I, Semedo L, Arrobas A. Omalizumab for severe asthma: beyond allergic asthma. Biomed Res Int. 2018;2018:3254094.PubMedPubMedCentralCrossRef Loureiro CC, Amaral L, Ferreira JA, Lima R, Pardal C, Fernandes I, Semedo L, Arrobas A. Omalizumab for severe asthma: beyond allergic asthma. Biomed Res Int. 2018;2018:3254094.PubMedPubMedCentralCrossRef
Metadata
Title
Long-term mepolizumab treatment reduces relapse rates in super-responders with eosinophilic granulomatosis with polyangiitis
Authors
Nami Masumoto
Chiyako Oshikata
Ryo Nakadegawa
Yuto Motobayashi
Reeko Osada
Saki Manabe
Takeshi Kaneko
Naomi Tsurikisawa
Publication date
01-12-2023
Publisher
BioMed Central
Published in
Allergy, Asthma & Clinical Immunology / Issue 1/2023
Electronic ISSN: 1710-1492
DOI
https://doi.org/10.1186/s13223-023-00801-7

Other articles of this Issue 1/2023

Allergy, Asthma & Clinical Immunology 1/2023 Go to the issue