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[Classification of systemic vasculatides].

Loïc Guillevin1, Christian Pagnoux, Philippe Guilpain

  • 1Centre de référence national, Plan maladies rares, vascularites et sclérodermie systémique, Hôpital Cochin, AP-HP, Université Paris 5 - René Descartes, Paris, France. loic.guillevin@cch.aphp.fr

Presse Medicale (Paris, France : 1983)
|April 6, 2007
PubMed
Summary

Vasculitis involves blood vessel inflammation, leading to blockages. Classifications like Chapel Hill distinguish conditions and highlight antineutrophil cytoplasm autoantibodies (ANCA) in specific vasculitides.

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Area of Science:

  • Rheumatology
  • Immunology
  • Pathology

Background:

  • Vasculitides are inflammatory conditions affecting blood vessel walls, causing stenosis or occlusion.
  • Current classifications include the 1990 American College of Rheumatology criteria and the 1994 Chapel Hill nomenclature.
  • The Chapel Hill nomenclature differentiated polyarteritis nodosa from microscopic polyangiitis and emphasized the role of antineutrophil cytoplasm autoantibodies (ANCA).

Purpose of the Study:

  • To review the classification of vasculitides.
  • To highlight the diagnostic significance of ANCA in primary systemic vasculitides.
  • To correlate ANCA specificities with specific small-vessel vasculitides.

Main Methods:

  • Review of existing literature and classification systems for vasculitis.

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  • Analysis of clinical presentations, histological features, and affected vessel size in vasculitis.
  • Examination of the association between ANCA and specific vasculitic entities.

    • Main Results:

    • Primary systemic vasculitides are clinically classified based on presentation, histology, and vessel size.
    • ANCA are associated with several small-vessel vasculitides.
    • Wegener's granulomatosis (90%), microscopic polyangiitis (80%), and Churg-Strauss syndrome (over 33%) show ANCA positivity, with specificities like anti-proteinase 3 and anti-myeloperoxidase.

    Conclusions:

    • Classification of vasculitis relies on a combination of clinical, histological, and serological markers.
    • ANCA are crucial diagnostic markers for specific types of small-vessel vasculitis.
    • Understanding ANCA patterns aids in differentiating and diagnosing conditions like Wegener's granulomatosis and microscopic polyangiitis.