Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[Wegener's granulomatosis].

Christian Pagnoux1, Luis Teixeira

  • 1Service de médecine interne, Centre de référence national maladies rares, vascularites nécrosantes et sclérodermies, Hôpital Cochin, AP-HP, Université Paris V - René Descartes, Paris, France. christian.pagnoux@cch.aphp.fr

Presse Medicale (Paris, France : 1983)
|March 17, 2007
PubMed
Summary

Wegener's granulomatosis, a systemic vasculitis, affects small to medium vessels and is often associated with cANCA anti-PR3 antibodies. While treatments achieve remission in most patients, the high relapse rate necessitates ongoing research into new therapies.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Chronic Rhinosinusitis and Nasal Polyps in Eosinophilic Granulomatosis With Polyangiitis: A Scoping Review.

International forum of allergy & rhinology·2026
Same author

Sleep disturbances in patients with vasculitis.

Sleep medicine·2026
Same author

Patients' perspectives of eosinophilic granulomatosis with polyangiitis within the context of a clinical trial.

EULAR rheumatology open·2026
Same author

IgG4-related disease presenting as an omental mass.

BMJ case reports·2026
Same author

Correction: Comprehensive immune profiling identifies alterations in adaptive and innate immune responses in granulomatosis with polyangiitis patients in remission.

Frontiers in immunology·2026
Same author

Impact of Neoadjuvant Chemotherapy Administration Time of Day on Pathological Response in Patients with Early Triple-Negative Breast Cancer.

Cancers·2026

Area of Science:

  • Immunology
  • Rheumatology
  • Nephrology

Background:

  • Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis impacting small to medium-sized vessels.
  • Cytoplasm-labeling antineutrophil cytoplasmic autoantibodies (cANCA) targeting proteinase 3 (PR3) are present in ~90% of WG patients.
  • WG incidence is increasing, primarily affecting adults aged 45-60, with complex immune mechanisms involving cANCA, neutrophils, lymphocytes, and cytokines.

Purpose of the Study:

  • To review the pathogenesis, clinical manifestations, diagnosis, and treatment of Wegener's granulomatosis.
  • To highlight the role of cANCA anti-PR3 antibodies in diagnosis and prognosis.
  • To discuss current treatment strategies and emerging therapeutic options.

Main Methods:

  • Literature review of Wegener's granulomatosis (WG).

Related Experiment Videos

  • Analysis of diagnostic criteria including clinical symptoms, cANCA anti-PR3 testing, and histology.
  • Evaluation of current treatment protocols (corticosteroids, cyclophosphamide) and novel agents (rituximab, anti-TNF-alpha).

    • Main Results:

    • WG diagnosis relies on clinical presentation, cANCA anti-PR3, and histology, with kidney biopsy offering prognostic value.
    • Current treatment induces remission in >85% of patients but is associated with a high relapse rate (~50% within 5 years).
    • New therapies like rituximab show promise for refractory cases.

    Conclusions:

    • WG management requires a multi-faceted approach combining immunosuppression and monitoring for relapse.
    • Understanding immune mechanisms is crucial for developing more effective and targeted therapies.
    • Ongoing research into novel agents may improve long-term outcomes and reduce relapse rates in WG.