Epidemiology and clinical features of systemic vasculitis

Clin Exp Nephrol. 2013 Oct;17(5):607-610. doi: 10.1007/s10157-013-0830-8. Epub 2013 Jul 11.


Different vasculitic syndromes present in different age groups. Immunoglobulin (Ig)A vasculitis and Kawasaki disease usually present in children whereas giant cell arteritis (GCA) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis usually present in the middle aged/elderly. In Northern Europe, granulomatosis with polyangiitis (GPA; Wegener's) is commoner than microscopic polyangiitis (MPA) and MPA is more common than eosinophilic granulomatosis with polyangiitis (EPGA; Churg-Strauss syndrome). In Southern Europe, MPA is commoner than GPA and in Japan MPA is much more common than GPA. Major differences exist worldwide in ANCA specificity which are not entirely related to different phenotypes. GPA, like GCA, has a cyclical pattern of onset suggesting possible infection as an aetiological agent. International studies have given important clues to possible aetiology including silica dust and infection and genetic influences, as shown by the recently published genome-wide association study which revealed that single-nucleotide polymorphisms associate more strongly with ANCA than clinical syndromes. A brief description of the main clinical features of ANCA-associated vasculitis is also given.

Publication types

  • Review

MeSH terms

  • Age of Onset
  • Genetic Predisposition to Disease
  • Humans
  • Phenotype
  • Predictive Value of Tests
  • Prognosis
  • Risk Factors
  • Systemic Vasculitis / classification
  • Systemic Vasculitis / diagnosis*
  • Systemic Vasculitis / epidemiology*
  • Systemic Vasculitis / genetics
  • Systemic Vasculitis / therapy
  • Terminology as Topic