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Review
. 2022 May 1;34(3):158-164.
doi: 10.1097/BOR.0000000000000873. Epub 2022 Apr 18.

Therapeutic advances in eosinophilic granulomatosis with polyangiitis

Julia A Ford  1 , Yaseen Aleatany, Ora Gewurz-Singer
Affiliations
Review

Therapeutic advances in eosinophilic granulomatosis with polyangiitis

Julia A Ford et al. Curr Opin Rheumatol. .

Abstract

Purpose of review: In recent years, therapeutic advances in eosinophilic granulomatosis with polyangiitis (EGPA) have changed our treatment paradigm. This review will summarize and discuss updates in management of EGPA, with a particular focus on biologic therapies.

Recent findings: The anti-interleukin (IL)-5 agent mepolizumab (the first FDA-approved drug specifically for EGPA) is effective in induction and maintenance of remission particularly in patients with predominantly asthma and allergic manifestations, though efficacy in ANCA-positive, vasculitic disease is unclear; additional anti-IL-5 agents are under study. Rituximab is currently recommended for remission induction in severe disease, particularly in ANCA-positive patients with vasculitic manifestations, though the supportive evidence is mostly observational. Evidence supporting use of traditional DMARDs and other biologic agents such as omalizumab remains limited and observational.

Summary: Although management of this heterogeneous disease remains challenging and unanswered questions remain, advances in biologics (particularly anti-IL-5 agents and an evolving interest in rituximab) have expanded our treatment armamentarium in EGPA.

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References

    1. Mohammad AJ. An update on the epidemiology of ANCA-associated vasculitis. Rheumatology (Oxford) 2020; 59: (Suppl 3): iii42–iii50.
    1. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol 2021; 73:1366–1383.
    1. Sablé-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143:632–638.
    1. Cohen P, Pagnoux C, Mahr A, 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; 57:686–693.
    1. Ribi C, Cohen P, Pagnoux C, Mahr A, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum 2008; 58:586–594.

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