Table 2

Examples of organ/life-threatening and not organ/life-threatening manifestations in patients with AAV

Examples of potentially organ/life-threatening manifestations*Examples of manifestations that are not ultimately organ/life-threatening*
GlomerulonephritisNasal and paranasal disease without bony involvement (erosion) or cartilage collapse or olfactory dysfunction or deafness
Pulmonary haemorrhageSkin involvement without ulceration
Meningeal involvementMyositis (skeletal muscle only)
Central nervous system involvementNon-cavitating pulmonary nodules
Retro-orbital diseaseEpiscleritis
Cardiac involvement
Mesenteric involvement
Mononeuritis multiplex
  • *These are just examples of typical disease manifestations and many other manifestations of AAV exist. Assessment of severity in the individual patient may differ (eg, scleritis can become organ threatening under certain circumstances).

  • AAV, antineutrophil cytoplasmic antibody-associated vasculitis.