Purpose of review: This study will provide an overview of the recent advances in treatment of chronic systemic vasculitis in childhood.
Recent findings: Advances in the treatment of chronic primary systemic vasculitis have significantly improved outcomes for patients in the last several decades. Conventional life-saving treatments like cyclophosphamide and corticosteroids have predominated as mainstay therapy for severe or extensive disease; however, evidence supporting the effectiveness of newer treatments such as rituximab and other biologic agents is increasing. Improved collaboration through international registries and multicentered initiatives has enabled increased research and generation of pediatric specific data. Such efforts are essential to the development of evidence-based treatment guidelines and validated assessment tools in the future.
Summary: Although most of our understanding about treatment of childhood vasculitis has been derived from adult data, pediatric data continue to accumulate. Evidence for newer treatments and alternative treatment strategies is continually evolving.