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[Wegener's granulomatosis and microscopic polyangiitis]

Christian Pagnoux1

  • 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
PubMed

Insights

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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