RARE VASCULITIC SYNDROMES

Vasculitis can and does occur in childhood. Apart from the common vasc ulitides (Henoch-Schonlein purpura, hypersensitivity angiitis and Kawa saki disease) there are a number of important but comparatively rare d isorders affecting children. These include macroscopic and microscopic polyarteritis, cutaneous polyarteritis, Wegener's granulomatosis, Chu rg-Strauss syndrome, primary angiitis of the central nervous system, h ypocomplimentaemic urticarial vasculitis, vasculitis associated with v arious connective tissue disorders, Takayasu's disease and vasculitis associated with conditions such as Behcet's syndrome, familial Mediter ranean fever and Cogan's syndrome. Distinguishing these conditions fro m other disorders is often difficult and requires clinical acumen and appropriate investigative procedures, With modern therapeutic agents i t is possible to implement appropriate therapy but in spite of this, t here remains a not inconsequential morbidity and mortality.