VASCULITIS

Vasculitis is the collective name for a group of serious, chronic auto-immune diseases in which the wall of the blood vessels gets inflamed.

 

Vasculitides (plural of vasculitis) can occur as a standalone disease of unknown cause or as a side effect of another disease. 

In the former case we speak of a primary vasculitis, in the latter case of a secondary vasculitis.

Sometimes a vasculitis only occurs in a certain part of the body. However, it often shows itself in a form that can occur throughout the body. We then speak of a systemic vasculitis.

Vasculitis International mainly focuses on the primary, systemic types of vasculitis.

 

 

Chapel Hill
Chapel Hill Classification

The so-called Chapel Hill classification defines the different types of vasculitis according to the size of the blood vessels affected.​

 

LARGE VESSEL VASCULITIS (LVV)
  • GCA Giant Cell Arteritis (Arteritis Temporalis)
  • ​TAK Takayasu Disease
MEDIUM VESSEL VASCULITIS (MVV)
  • PAN Polyarteritis Nodosa
  • ​KD Kawasaki Disease
SMALL VESSEL VASCULITIS (SVV)
  • ​GPA Granulomatosis with Polyangiitis (Wegener’s)
  • EGPA Eosinofile Granulomatosis with Polyangiitis
  • MPA Microscopic Polyangiitis

(collectively referred to as ANCA Associated SVV)

  • ​Anti GBM Disease (Good Pasture)
  • ​IgA vasculitis (Henoch-Schönlein)
  • ​CV Cryoglobulinemic Vasculitis
  • Anti C1q vasculitis

(collectively referred to as Immune Complex SVV)

VARIABLE VESSEL VASCULITIS
  • Behçet Disease
  • ​Cogan’s Syndrome
SPECIFIC ORGAN VASCULITIS

Cutane leukocytoclastic angiitis (CLA), Cutane Arteritis, Primary CNS vasculitis, Isolated Otitis, and other.