Table 1.
Characteristics of different types of vasculitis
| Type of vasculitis | Age tropism | Affected vessels | Pathology | Major clinical features |
|---|---|---|---|---|
| Large vessel vasculitis | ||||
| Takayasu arteritis | <50 years | Aorta and its major branches | Granulomatous arteritis | Limb claudication, pulse loss and systemic illness |
| Giant cell arteritis | >60 years | Predilection for the branches of the carotid and vertebral arteries, especially the temporal arteries | Granulomatous arteritis with giant cells | Headache, polymyalgia rheumatica, constitutional symptoms, visual disturbance and limb claudication |
| Medium vessel vasculitis | ||||
| Polyarteritis nodosa | None | Medium or small arteries | Necrotizing arteritis | Mononeuritis multiplex, testicular pain and skin ulceration; associated with HBV infection; not associated with glomerulonephritis or ANCAs |
| Kawasaki disease | <5 years | Medium and small arteries, especially coronary arteries | Pan arteritis | Mucocutaneous lymph node syndrome and coronary artery aneurysms |
| Small vessel vasculitis | ||||
| Granulomatosis with polyangiitis | More common with increasing age | Small vessels | Necrotizing granulomatous inflammation | Affects the upper and lower respiratory tract; necrotizing glomerulonephritis is common; associated with PR3-ANCAs |
| Microscopic polyangiitis | More common with increasing age | Small vessels | Necrotizing vasculitis with few or no immune deposits and no granulomata | Necrotizing glomerulonephritis is very common; pulmonary capillaritis often occurs; associated with MPO-ANCAs |
| Eosinophilic granulomatosis with polyangiitis | More common with increasing age | Small to medium vessels | Eosinophil-rich and necrotizing granulomatous inflammation | Affects the respiratory tract and is associated with asthma and eosinophilia; ANCAs are more frequent when glomerulonephritis is present |
| IgA vasculitis | <16 years | Predominantly capillaries, venules or arterioles | Vasculitis with IgA1-dominant immune deposits | Often involves skin, joints and gastrointestinal tract; glomerulonephritis indistinguishable from IgA nephropathy can occur |
| Hypocomplementaemic urticarial vasculitis | 40–60 years | Predominantly capillaries, venules or arterioles | Vasculitis | Urticaria, glomerulonephritis, arthritis, obstructive pulmonary disease and ocular inflammation are common; anti-C1q antibodies are present |
| Other | ||||
| Behçet syndrome | More common during the second and third decades | Aorta, arteries and veins of all sizes, including cerebral venous sinuses | Non-granulomatous, predominantly neutrophilic inflammation | Oral and genital ulcers, papulopustular and erythema nodosum-like lesions, arthritis, uveitis and vascular involvement; central nervous system and gastrointestinal system involvement is less frequent |
ANCA, antineutrophil cytoplasmic antibody; C1q, complement protein C1q; HBV, hepatitis B virus; MPO, myeloperoxidase; PR3, proteinase 3.