4.1 Review

Biologics for eosinophilic granulomatosis with polyangiitis

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Review Medicine, General & Internal

Biologic Therapies for Severe Asthma

Guy G. Brusselle et al.

Summary: Biologic therapies may be necessary to reduce the disease burden and risks for severe asthma patients. This review discusses the mechanisms, efficacy, and safety of biologics for severe asthma.

NEW ENGLAND JOURNAL OF MEDICINE (2022)

Review Rheumatology

Therapeutic advances in eosinophilic granulomatosis with polyangiitis

Julia A. Ford et al.

Summary: This review summarizes the recent advances in the management of eosinophilic granulomatosis with polyangiitis (EGPA), with a focus on the use of biologic therapies. The anti-interleukin (IL)-5 agent mepolizumab has shown efficacy in inducing and maintaining remission, particularly in patients with asthma and allergic manifestations. Rituximab is recommended for remission induction in severe disease, especially in ANCA-positive patients with vasculitic manifestations. However, the evidence for the use of traditional DMARDs and other biologic agents is limited. Advances in biologics have expanded the treatment options for EGPA.

CURRENT OPINION IN RHEUMATOLOGY (2022)

Article Rheumatology

Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study

Alessandra Bettiol et al.

Summary: Mepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks proved effective in treating EGPA, with no significant difference between the two doses. Complete response rates reached 30.4% at 12 months and 35.7% at 24 months, with some patients experiencing exacerbations in asthma and ENT.

ARTHRITIS & RHEUMATOLOGY (2022)

Review Pharmacology & Pharmacy

Perspectives on the Efficacy of Benralizumab for Treatment of Eosinophilic Granulomatosis With Polyangiitis

Yasuhiko Koga et al.

Summary: Benralizumab, an IL-5 antibody biologic, has shown efficacy in treating severe asthma and EGPA. It has the ability to bind to IL-5 alpha receptors and effectively eliminate eosinophils. Studies have demonstrated that benralizumab treatment can reduce oral corticosteroid doses and achieve remission in some refractory cases. It is considered safe and tolerable in different age groups.

FRONTIERS IN PHARMACOLOGY (2022)

Review Immunology

The Th17 Pathway in Vascular Inflammation: Culprit or Consort?

Marie Robert et al.

Summary: The involvement of IL-17A in autoimmune and inflammatory diseases has led to research on therapeutic strategies to block the Th17 pathway. IL-17A plays a positive role in inflammation, coagulation, thrombosis, and cardiovascular events, while its involvement in allograft rejection and vasculitis has been confirmed. Anti-IL-17 therapy may be a promising strategy for vasculitis treatment, but lessons from rheumatoid arthritis and multiple sclerosis need to be learned.

FRONTIERS IN IMMUNOLOGY (2022)

Article Biochemistry & Molecular Biology

Gut Microbiota and Associated Mucosal Immune Response in Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Elena Niccolai et al.

Summary: In this study, the gut microbiota composition and intestinal immune response in EGPA patients were characterized. The results showed an enrichment of potential pathobionts in EGPA patients, particularly in those with active disease, and a positive correlation between disease activity and intestinal T-cell levels. Immunosuppression in patients may reduce the levels of potential pathobionts and alleviate inflammation.

BIOMEDICINES (2022)

Meeting Abstract Rheumatology

EFFICACY OF MEPOLIZUMAB IN PATIENTS WITH EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS AND A VASCULITIC PHENOTYPE

B. Terrier et al.

ANNALS OF THE RHEUMATIC DISEASES (2022)

Review Immunology

The Therapeutic Potential for Targeting Group 2 Innate Lymphoid Cells in Asthma

Takahiro Matsuyama et al.

Summary: T helper type 2 cells and group 2 innate lymphoid cells play a crucial role in asthma, particularly in airway eosinophilic inflammation. These cells are activated by cytokines released by airway epithelial cells and produce high levels of type 2 cytokines. ILC2s induce airway inflammation independently of antigens and may contribute to steroid resistance. Biologics targeting these cytokines and/or their receptors have shown promise in reducing asthma exacerbations and improving lung function and quality of life in asthmatic patients.

FRONTIERS IN IMMUNOLOGY (2022)

Review Allergy

The new indications for biologicals in type 2 diseases: perspectives

Diego Bagnasco et al.

Summary: Literature review shows that drugs targeting type 2 inflammation have proven effective in treating asthma and nasal polyposis, and have promising potential in the treatment of eosinophilic esophagitis, particularly with the use of anti-IL-5/IL-5 receptor and IL-4 receptor antibodies. In the case of eosinophilic granulomatosis with polyangitis (EGPA), mepolizumab (MEP) has been approved by FDA and EMA due to positive trial results and real-life application. The use of these drugs is a significant advancement in the treatment of complex diseases, providing alternative options to immunosuppressants and systemic corticosteroids.

CURRENT OPINION IN ALLERGY AND CLINICAL IMMUNOLOGY (2022)

Review Rheumatology

Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management

Giorgio Trivioli et al.

Summary: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare and complex disease driven by the interaction of genetic and environmental factors, with genetic associations varying based on ANCA specificity and contributing to differences in clinical phenotype and treatment response.

NATURE REVIEWS RHEUMATOLOGY (2022)

Article Medicine, General & Internal

Real-life impact of uncontrolled severe asthma on mortality and healthcare use in adolescents and adults: findings from the retrospective, observational RESONANCE study in France

Nicolas Roche et al.

Summary: This study confirms the significant burden of uncontrolled severe asthma in terms of mortality, morbidity, and healthcare resource consumption compared to other asthma patients and the general population, highlighting the importance of appropriate management in this high-risk population.

BMJ OPEN (2022)

Review Allergy

Antineutrophil Cytoplasmic Antibodies and Organ-Specific Manifestations in Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis

Hua-Ching Chang et al.

Summary: The study investigated the association of ANCA status with organ-specific manifestations in EGPA, revealing that compared with ANCA-negative patients, ANCA-positive patients had higher risks of peripheral neuropathy, renal involvement, and cutaneous purpura, and lower risks of pulmonary infiltrates and cardiac involvement. The study also found no significant association of ANCA status with asthma and involvements of the central nervous system, gastrointestinal tract, or skin.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE (2021)

Article Rheumatology

Use of Biologics to Treat Relapsing and/or Refractory Eosinophilic Granulomatosis With Polyangiitis: Data From a European Collaborative Study

Alice Canzian et al.

Summary: The study suggests that RTX may be effective in treating relapses of EGPA vasculitis. MEPO is highly effective with a good safety profile in patients with GC-dependent asthma.

ARTHRITIS & RHEUMATOLOGY (2021)

Letter Allergy

Mepolizumab 100 mg in severe asthmatic patients with EGPA in remission phase

Marco Caminati et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE (2021)

Review Rheumatology

Elevated Serum IgG4 Was Found in Eosinophilic Granulomatosis With Polyangiitis

Ziyan Wu et al.

Summary: The study found elevated levels of IgG4 in Chinese patients with EGPA, with the EGPA group showing significantly higher levels of IgG4 and IgG4/IgG ratio compared to other groups and healthy controls. Further research is needed to determine the pathological role of IgG4 and IgG4 antineutrophil cytoplasmic antibodies in EGPA.

JCR-JOURNAL OF CLINICAL RHEUMATOLOGY (2021)

Article Rheumatology

Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Matthias Papo et al.

Summary: PR3-ANCA EGPA patients differ from MPO-ANCA and ANCA-negative patients in clinical presentation, but share similarities with granulomatosis with polyangiitis, suggesting it may be a distinct form of PR3-ANCA-associated vasculitis.

RHEUMATOLOGY (2021)

Review Medicine, General & Internal

Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology

Filippo Fagni et al.

Summary: EGPA is a rare multisystemic disease characterized by vessel inflammation and eosinophilic proliferation. It is divided into different phenotypes based on ANCA status, with diagnosis and treatment implications.

FRONTIERS IN MEDICINE (2021)

Letter Rheumatology

Microarray evaluation of allergen-specific IgE in eosinophilic granulomatosis with polyangiitis

Federica Bello et al.

ANNALS OF THE RHEUMATIC DISEASES (2021)

Article Medicine, General & Internal

Tezepelumab in Adults and Adolescents with Severe, Uncontrolled Asthma

Andrew Menzies-Gow et al.

Summary: Tezepelumab, a monoclonal antibody targeting thymic stromal lymphopoietin, effectively reduces asthma exacerbations in patients with severe, uncontrolled asthma, leading to improved lung function, asthma control, and quality of life.

NEW ENGLAND JOURNAL OF MEDICINE (2021)

Article Rheumatology

Occupational Exposures and Smoking in Eosinophilic Granulomatosis With Polyangiitis: A Case-Control Study

Federica Maritati et al.

Summary: Exposure to silica, farming, or organic solvents is associated with an increased risk of Eosinophilic Granulomatosis with Polyangiitis (EGPA), while smoking is associated with a lower risk. The association of silica and farming is primarily aligned with ANCA-positive EGPA, while the association of smoking status and organic solvents is primarily aligned with ANCA-negative EGPA.

ARTHRITIS & RHEUMATOLOGY (2021)

Review Medicine, General & Internal

ANCA-Associated Vasculitis: An Update

Salem Almaani et al.

Summary: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. AAV involves multiple organ systems clinically, including the lungs, kidneys, skin, and nervous system. The prognosis of AAV has significantly improved due to advances in understanding its pathogenesis and treatment options.

JOURNAL OF CLINICAL MEDICINE (2021)

Article Medicine, Research & Experimental

A role for IL-33-activated ILC2s in eosinophilic vasculitis

Maya E. Kotas et al.

Summary: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and poorly understood disease characterized by elevated levels of TSLP, IL-25, and soluble ST2 in patients, alongside reduced circulating ILC2s. A mouse model study revealed the important roles of IL-33, ILC2s, and IL4R alpha signaling in the pathogenesis of EGPA.

JCI INSIGHT (2021)

Article Rheumatology

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Sharon A. Chung et al.

Summary: This article provides evidence-based recommendations and expert guidance for the management of AAV, including 26 recommendations and 5 ungraded position statements for GPA/MPA and 15 recommendations and 5 ungraded position statements for EGPA. The guidance aims to assist healthcare professionals in treating these diseases.

ARTHRITIS & RHEUMATOLOGY (2021)

Letter Allergy

Reply to Mepolizumab in patients with eosinophilic granulomatosis with polyangiitis in remission: What is the right dose?

Marco Caminati et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE (2021)

Review Rheumatology

Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis of Test Accuracy and Benefits and Harms of Common Treatments

Jason M. Springer et al.

Summary: Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of ANCA-associated vasculitis characterized by asthma and peripheral eosinophilia. This systematic review evaluates the diagnosis and management of EGPA, highlighting the accuracy of diagnostic tests and the benefits/toxicities of various treatment options.

ACR OPEN RHEUMATOLOGY (2021)

Letter Allergy

Eosinophilic granulomatosis with polyangiitis evolution during severe eosinophilic asthma treatment with benralizumab

Alojzija Hocevar et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE (2020)

Review Allergy

Biologics for the Treatments of Allergic Conditions Severe Asthma

Marco Caminati et al.

IMMUNOLOGY AND ALLERGY CLINICS OF NORTH AMERICA (2020)

Article Allergy

Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis

Naomi Tsurikisawa et al.

CLINICAL AND EXPERIMENTAL ALLERGY (2018)

Article Medicine, General & Internal

Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis

M. E. Wechsler et al.

NEW ENGLAND JOURNAL OF MEDICINE (2017)

Article Respiratory System

Respiratory manifestations of eosinophilic granulornatosis with polyanglitis (Churg-Strauss)

Vincent Cottin et al.

EUROPEAN RESPIRATORY JOURNAL (2016)

Article Rheumatology

Eotaxin-3 in Churg-Strauss syndrome: a clinical and immunogenetic study

Jochen Zwerina et al.

RHEUMATOLOGY (2011)