According to the 2012 Chapel Hill Consensus Conference, vasculitides are classified as follows.
| I | Vasculitis of the small vessels (small-vessel vasculitides) | |
| ANCA-associated vasculitides (AAV). | ||
| 1 | Granulomatosis with polyangiitis (GPA)[formerly: Wegener’s granulomatosis]. | |
| 2 | Eosinophilic granulomatosis with polyangiitis (EGPA)[formerly: Churg-Strauss syndrome (CSS)] | |
| 3 | Microscopic polyangiitis (MPA) | |
| Non-ANCA associated | ||
| 4 | Anti-GBM disease[formerly: Goodpasture’s syndrome]. | |
| 5 | Schönlein-Henoch purpura[new: IgA vasculitis (IgAV)] | |
| 6 | Cryoglobulinemic vasculitis (CV) (vasculitis associated with essential cryoglobulinemia). | |
| 7 | Hypocomplementemic urticarial vasculitis (HUV, anti-C1q vasculitis). | |
| II | Vasculitis of medium-sized vessels (vasculitides of medium-sized vessels) | |
| 1 | Classic panarteritis | |
| 2 | Kawasaki disease (MCLS; Kawasaki syndrome) | |
| III | Large vessel vasculitis (large vessel vasculitides) | |
| 1 | Giant cell arteritis | |
| 2 | Takayasu arteritis | |
| IV | Vasculitis of variable vessel size | |
| Behçet’s disease | ||
| Cogan syndrome | ||
| V | Single-organ vasculitis | |
| Cutaneous leukocytoclastic angiitis | ||
| Cutaneous arteritis | ||
| More | ||
| VI | Systemic vasculitis | |
| Lupus vasculitis | ||
| Rheumatoid vasculitis | ||
| More | ||
| VII | Secondary vasculitis | |
| Hepatitis C-associated | ||
| Hepatitis B-associated | ||
| Drug-associated | ||
| Tumor-associated |
Activity stages of ANCA-associated vasculitides (AAV) – EUVAS definition.
| Activity stage | Definition |
| Localized stage | Upper and/or lower respiratory tract without systemic manifestation, without B symptoms, not organ-threatening1 |
| Early systemic stage | All organ involvement possible, not life-threatening or organ-threatening2 |
| Generalization stage | Renal involvement (kidney involvement) or other organ-threatening manifestation (serum creatinine < 500 µmol/l (5.6 mg/dl))3 |
| Severe, vital-threatening generalization stage | Renal failure or other organ failure (creatinine > 500 µmol/l (5.6 mg/dl))3 |
| Refractory stage | Progressive disease, refractory to standard therapy (glucocorticoids, cyclophosphamide) |
Legend
- 1ANCA often negative
- 2ANCA negative or positive
- 3ANCA almost always positive
B symptomatology
- Unexplained, persistent or recurrent fever (> 38 °C).
- Night sweats (wet hair, soaked sleepwear).
- Unwanted weight loss (> 10% percent of body weight within 6 months).
Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, and eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome (CSS), can also be classified according to ACR criteria* (see under the corresponding disease).
