Kawasaki disease and Henoch-Schönlein purpura - 10 years' experience of childhood vasculitis at a university hospital in Taiwan

Abstract

Background/purpose: To investigate the clinical manifestations, disease activity and prognosis in different types of vasculitis.

Methods: The charts of pediatric patients with vasculitis diagnosed from December 1997 to December 2007 were retrospectively reviewed. The first clinical manifestations and laboratory results were recorded at the time of diagnosis, and outcome evaluations with history of flare-ups were analyzed.

Results: A total of 508 vasculitis patients were included in this study, of whom 124 had Henoch-Schönlein purpura (HSP), and 351 had Kawasaki disease (KD). Hematuria was observed in 79% of recurrent HSP patients at the time of diagnosis, and was associated with an increased risk of relapse (p = 0.000). In Kawasaki disease, the clinical symptoms with erythematous changes in Bacille Calmette-Guérin scars and coronary artery dilatation were more prominent in patients younger than 1 year old, and lymphadenopathy was more common in patients older than 1 year old (p = 0.001). The risk of coronary dilatation was significant in the patients with an initial presentation of thrombocytosis, and greater in patients younger than 1 year old (p = 0.027). Thrombocytopenia was more prominent in vasculitis-associated autoimmune diseases. Marked lymphocytosis with increased C-reactive protein levels was significantly noted in urticarial vasculitis patients compared with HSP patients in multivariate logistic regression analysis.

Conclusion: Vasculitis disease activity and prognosis were associated with initial laboratory results and clinical manifestations. Further large-scale clinical trials are warranted to validate these findings.