The Vasculitides
© Dirk Biddle
3.1 Primary Vasculitides
Primary vasculitides are probably best considered as vasculitis conditions that are diseases in their own right with no defined preceding or accompanying disease. Primary vasculitides have often been described as having “no cause” or as arising “out of the blue”, but these terms are misleading. Causal elements and mechanisms have been postulated and found for some primary vasculitides (e.g. the link between smoking and Buerger’s disease). It is therefore clear (clinically and logically) that primary vasculitis conditions do (and indeed must) have causes, it is just that in many cases that cause has not yet been discovered. Many primary vasculitides can thus be described as idiopathic (literally meaning of unknown cause).
There are thirteen recognised primary vasculitides:
- Behcet’s disease
- Buerger’s disease
or thromboangiitis obliterans
- Central Nervous System vasculitis (CNS vasculitis)
or Primary Angiitis of the Central Nervous System (PACNS)
- Eosinophilic Granulomatosis with Polyangitis (EGPA)
(formerly Churg-Strauss Syndrome)
- Cryoglobulinemia
or cryoglobulinaemic vasculitis (CV)
- Cutaneous Leukocytoclastic Angiitis
or leukocytoclastic vasculitis
or hypersensitivity vasculitis
- Giant Cell Arteritis (GCA)
or temporal arteritis
- IgA Vasculitis
formerly Henoch-Schönlein Purpura (HSP)
- Kawasaki Disease
or mucocutaneous lymph node syndrome
- Microscopic polyangiitis (MPA)
or microscopic Polyarteritis
- Polyarteritis Nodosa (PAN)
or panarteritis nodosa
- Takayasu’s Arteritis (TAK)
or pulseless disease
- Granulomatosis with Polyangiitis (GPA)
formerly Wegener’s granulomatosis
