Incidence and clinical features of paediatric vasculitis in Eastern China: 14-year retrospective study, 1999-2013.

objectivesTo determine the incidence and clinical features of paediatric primary vasculitis in patients from one centre in Eastern China.

methodsMedical records of paediatric patients diagnosed with primary vasculitis between January 1999 and December 2013 were retrospectively reviewed. For Henoch-Schönlein purpura (HSP) and Kawasaki disease (KD), patients included in the analyses had data available for the previous 5 years.

resultsIn total, 1896 patients were identified, of whom 1100 had HSP, 760 had KD, 23 had Takayasu arteritis, five had polyarteritis nodosa, four had cutaneous polyarteritis, three had Behçet's disease and one had microscopic polyangiitis. Of the 615 patients with HSP included in the analyses, 49.8% had HSP nephritis (for 90% of whom it occurred within 1 week of disease onset). Of the 470 patients with KD included in the analyses, 13.8% were diagnosed with incomplete KD and 29.0% had a concurrent coronary artery lesion. For the 23 patients with Takayasu arteritis, the common clinical symptoms were hypertension, asphygmia/weak pulse and heart failure; only one of these patients had been diagnosed at an early disease stage. The five patients with polyarteritis nodosa received immunosuppressant therapy following diagnosis. Other vasculitides were uncommon.

conclusionsThe most common primary vasculitides in this population of children from Eastern China were HSP and KD; other vasculitides were rare. Paediatricians should be suspicious of vasculitis when there is evidence of systemic inflammation and multisystem disease that cannot be explained by one specific disorder.