Immune Vasculitis

Julia D Flint, University of Birmingham, Birmingham, UK
Caroline OS Savage, University of Birmingham, Birmingham, UK

Published online: March 2010

DOI: 10.1002/9780470015902.a0002150.pub2

Vasculitis comprises a broad group of diseases characterized by inflammation and necrosis in the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The conditions are usually classified according to the predominant size of the vessels involved. A wide range of immunopathological mechanisms have been implicated in the aetiology of the vasculitides and a subset of small-vessel vasculitides are strongly associated with antineutrophil cytoplasmic antibodies (ANCA). The distribution of blood vessel involvement varies considerably, resulting in a heterogeneous clinical presentation, often involving multiple organ systems. This makes both classification and diagnosis of these diseases challenging. Clinical management is hindered by our limited understanding of the immunopathogenesis of these conditions. However, research in this area is active, many treatment trials are in progress and the morbidity and mortality has fallen in recent years.

Key concepts:

  • Vasculitis may be ‘idiopathic’ or ‘primary’ but it is important to rule out common mimics of vasculitis and the many underlying conditions that can induce a ‘secondary’ vasculitis.
  • Vasculitides are usually classified according to the predominant size of the vessels involved, but there is ongoing debate as to the most accurate and clinically useful way to classify the conditions.
  • Immunopathological mechanisms are diverse, with roles for both humoral and cellular immune mechanisms.
  • A subset of small-vessel vasculitides are strongly associated with antineutrophil cytoplasmic antibody (ANCA) and this is often used as a biomarker to aid diagnosis.
  • There is strong evidence for a pathogenic role of ANCA in the ‘ANCA associated vasculitides’, but the extent of this continues to be debated.
  • Vasculitis is usually considered a ‘systemic’ disease with the propensity to affect blood vessels throughout the body, but individual clinical presentations may be limited to specific organ systems.
  • Many patients initially experience a prodrome of nonspecific symptoms such as malaise, fatigue, fever, night sweats or weight loss.
  • The prevalence of vasculitis is increasing. This may be due to the increased rates of diagnosis, due to clinical suspicion and use of biomarkers such as ANCA; increased survival post-diagnosis or a true increased incidence.
  • Further research into the underlying pathological processes is vital to identifying potential treatment targets.
  • Many trials are currently in progress and will contribute to the improving rates of morbidity and mortality in systemic vasculitis.

Keywords: inflammation; endothelium; autoantibodies; immunopathology; antineutrophil cytoplasmic antibody

Figure 1. Initial investigations may support a diagnosis of vasculitis or help to exclude common mimics or secondary vasculitis syndromes. Actual investigations performed will depend on the individual patient and the local expertise. Adapted from Jayne (2009) with permission from Elsevier.
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    Morgan MD, Harper L, Williams J and Savage C (2006) Anti-neutrophil cytoplasm associated glomerulonephritis. Journal of the American Society of Nephrology 17: 1224–1234.
    Savage CO, Harper L, Cockwell P, Adu D and Howie AJ (2000) ABC of arterial and vascular disease: vasculitis. British Medical Journal 320(7245): 1325–1328.
    Savage COS (2006) Medium vessel vasculitides. Medicine 34(11): 464–467.
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Related Sub-Topics:

Inflammation | Diagnostics, Therapeutics and Methods | Allergy and Hypersensitivity
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Article Title: Immune Vasculitis
 
How to Cite close
Flint, Julia D, and Savage, Caroline OS(Mar 2010) Immune Vasculitis. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0002150.pub2]