Introduction to systemic vasculitides

This topic provides general advice on some of the vasculitides, a heterogeneous group of uncommon diseases ranging from self-limited cutaneous vasculitides to catastrophic life- and organ-threatening systemic vasculitides. Specific systemic vasculitides are discussed in more detail in separate topics, including:

The cutaneous vasculitides, leukocytoclastic vasculitis, and urticarial vasculitis, are addressed in the Dermatology guidelines.

The systemic vasculitides are thought to have an autoimmune pathogenesis. Vasculitis (inflammation of the blood vessel wall) can result in occlusion, stenosis or aneurysm of affected blood vessels. Damage to the blood vessels leads to organ damage through haemorrhage or infarction. The kidneys and lungs are most often affected by vasculitis, and the severity and extent of their involvement is closely related to the disease prognosis.

Key points about systemic vasculitides are listed in Key points about systemic vasculitides.

The specific systemic vasculitides can be characterised by the size and type of affected blood vessels; see Clinical features of systemic vasculitides for clinical features of systemic vasculitides. Serology and organ involvement are increasingly recognised in the classification of these conditions, which is evolving.
Figure 1. Key points about systemic vasculitides
  • Systemic vasculitides are serious diagnoses that can be organ-threatening, body system–threatening or life-threatening, with a high mortality if not managed aggressively from the outset.
  • These conditions need to be managed urgently by a specialist.
  • Management typically requires high-dose systemic corticosteroid therapy.
  • Treatment typically comprises an initial induction phase, aimed at remission of disease activity, followed by subsequent maintenance of remission.