Vasculitis
Vasculitides are a group of heterogenous disorders that have destructive inflammation within blood vessel walls.
Classification of Vasculitis
The Chapel Hill Consensus on nomenclature has at least 27 forms of primary vasculitis. Classification of vasculitides are based on:
- Size of blood vessel involvement
- Large - aorta, primary branches, and vessels not within an organ
- Medium - main visceral arteries and their branches
- Small - smaller intraparenchymal arteries, arterioles, capillaries, and venules
- Granulomatous inflammation or immune complex deposition
- Disease-specific autoantibodies such as ANCA Associated Vasculitis
- Tropism for certain organs
- Age, sex, ethinicity
Primary Vasculitides
| Group | Examples |
|---|---|
| Large-Vessel | Takayasu arteritis Giant cell arteritis |
| Medium-Vessel | Polyarteritis nodosa Kawasaki disease Buerger disease |
| Small-Vessel | ANCA Associated Vasculitis Immune complex small-vessel vasculitis Anti-GBM disease Cryoglobulinemic vasculitis IgA vasculitis Hypocomplementic urticarial vasculitis |
| Variable-Vessel | Behcet syndrome Cogan syndrome |
| Single-Organ | Cutaneous leukocytoclastic angiitis Cutaneous arteritis Primary CNS vasculitis Isolated aortitis |
| Systemic Disease | Lupus vasculitis Rheumatoid vasculitis Sarcoid vasculitis IgG4-related aortitis |
| Probable Etiology | HCV related cryoglobulinemic vasculitis HBV associated vasculitis Syphilis associated vasculitis Drug-associated-immune complex vasculitis Drug-associated ANCA vasculitis Cancer-associated vasculitis |