alexa Churg-Strauss syndrome.


Journal of Vasculitis

Author(s): Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M,

Abstract Share this page

Abstract Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS≥1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative. Copyright © 2015 Elsevier B.V. All rights reserved. This article was published in Autoimmun Rev and referenced in Journal of Vasculitis

Relevant Expert PPTs

Relevant Speaker PPTs

  • Hana Zelenkova
    Therapy with immune modulators (cyclosporine A) in dermatology (focusing on psoriasis, atopic eczema, allergic vasculitis, and chronic urticaria)
    PPT Version | PDF Version

Recommended Conferences

Peer Reviewed Journals
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2017-18
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

Agri, Food, Aqua and Veterinary Science Journals

Dr. Krish

1-702-714-7001 Extn: 9040

Clinical and Biochemistry Journals

Datta A

1-702-714-7001Extn: 9037

Business & Management Journals


1-702-714-7001Extn: 9042

Chemical Engineering and Chemistry Journals

Gabriel Shaw

1-702-714-7001 Extn: 9040

Earth & Environmental Sciences

Katie Wilson

1-702-714-7001Extn: 9042

Engineering Journals

James Franklin

1-702-714-7001Extn: 9042

General Science and Health care Journals

Andrea Jason

1-702-714-7001Extn: 9043

Genetics and Molecular Biology Journals

Anna Melissa

1-702-714-7001 Extn: 9006

Immunology & Microbiology Journals

David Gorantl

1-702-714-7001Extn: 9014

Informatics Journals

Stephanie Skinner

1-702-714-7001Extn: 9039

Material Sciences Journals

Rachle Green

1-702-714-7001Extn: 9039

Mathematics and Physics Journals

Jim Willison

1-702-714-7001 Extn: 9042

Medical Journals

Nimmi Anna

1-702-714-7001 Extn: 9038

Neuroscience & Psychology Journals

Nathan T

1-702-714-7001Extn: 9041

Pharmaceutical Sciences Journals

John Behannon

1-702-714-7001Extn: 9007

Social & Political Science Journals

Steve Harry

1-702-714-7001 Extn: 9042

© 2008-2017 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version