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Updated: Jul 4, 2026
06:35
An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
[Wegener's granulomatosis and microscopic polyangiitis]
1Centre de référence maladies auto-immunes et maladies systémiques rares, vascularites nécrosantes et sclérodermie systémique, pôle de médecine, hôpital Cochin, Université Paris-5, Paris. christian.pagnoux@cch.aphp.fr
La Revue Du Praticien
|June 6, 2008
Summary
Wegener's granulomatosis and microscopic polyangiitis are small vessel vasculitides. Diagnosis relies on ANCA testing and histology, with treatment involving immunosuppressants and corticosteroids.
Area of Science:
- Rheumatology
- Nephrology
- Pulmonology
Background:
- Wegener's granulomatosis and microscopic polyangiitis are systemic necrotizing vasculitides affecting small vessels.
- Prevalence ranges from 24-157 cases per million, with onset typically between 40-60 years.
- Key features include respiratory, renal, and neurological involvement, along with skin manifestations.
Purpose of the Study:
- To review the epidemiology, clinical manifestations, diagnosis, and treatment of Wegener's granulomatosis and microscopic polyangiitis.
- To highlight the role of Antineutrophil Cytoplasm Autoantibodies (ANCA) in diagnosis.
- To discuss current therapeutic strategies and long-term outcomes.
Main Methods:
- Literature review of Wegener's granulomatosis and microscopic polyangiitis.
- Analysis of diagnostic criteria, including serological markers (ANCA) and histological findings.
- Summary of established treatment protocols and emerging therapies.
Main Results:
- ANCA against proteinase 3 (PR3) found in 90% of Wegener's granulomatosis cases; ANCA against myeloperoxidase (MPO) in >60% of microscopic polyangiitis cases.
- Histological differentiation is possible based on granulomatous inflammation in Wegener's granulomatosis.
- Standard therapy includes corticosteroids and cyclophosphamide, with azathioprine or methotrexate for maintenance, achieving >80% 10-year survival but with frequent relapses.
Conclusions:
- Wegener's granulomatosis and microscopic polyangiitis require prompt diagnosis and management.
- Effective treatment improves survival rates, but long-term monitoring is essential due to relapse risk.
- Further research is needed to define the role of biologics like rituximab.
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