Primary systemic vasculitis in children in Estonia: a retrospective study from 2001-2010

Results 197 new cases of PSV were diagnosed during these years – HSP in 154, KD in 35, Churg-Staruss syndrome in 2, and polyarteritis nodosa in 3 cases, respectively. Wegener granulomatosis, microscopic polyangiitis and hypocomplementaemic urticarial vasculitis were all diagnosed in one patient. The age distribution ranged from 3 months to 16 years, in cases of KD – from 2 months to 13 years. 38% of all patients with HSP received short term corticosteroids. 91,4 % patients with KD received high dose intravenous immunoglobulin. Three children died during the follow up period (Wegener granulomatosis, polyarteritis nodosa, microscopic polyangiitis). Conclusions We determined the number of new cases of PSV within a hospital based population in Estonia. Despite the small population of Estonia we had the overall variety of PSV. HSP was the most common vasculitis, followed by KD. Other PSV were very rare in children in Estonia.