Objective: To identify the need for, and feasibility of, establishing a web-based USA/Canadian registry of children with chronic systemic vasculitis--an otherwise insufficiently studied population.
Methods: Physician members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA; n = 126) were invited to complete 2 surveys exploring vasculitis-related experience, beliefs about childhood versus adult vasculitis, and commitment to contribute patients to a prospective registry. Diagnoses included Wegener's granulomatosis (WG), childhood polyarteritis nodosa, microscopic polyangiitis (MPA), Takayasu's arteritis, primary angiitis of the central nervous system (PACNS), vasculitis, and unclassified vasculitis.
Results: One or both surveys were completed by 102 (81%) physicians. Almost half of first-survey respondents had been in practice for > 15 years. Collective estimated lifetime experience was >1500 patients (WG and unclassified vasculitis were the most common diagnoses). Three hundred seventeen children with vasculitis were seen in the year preceding the survey, with most physicians seeing only 2-5 patients. The majority of respondents believed that childhood vasculitis differed from adult disease, particularly with respect to classification criteria and disease activity markers. Fifty-nine members committed to contribute 2 years' data (approximately 120 patients) to a pilot registry limited to time of diagnosis, focusing on WG, MPA, Churg-Strauss syndrome, PACNS, and unclassified vasculitis.
Conclusion: We obtained overwhelming consensus from an experienced body of pediatric rheumatologists on the need to study childhood-onset vasculitis independently from adult disease, together with commitment from sufficient members to prospectively contribute 2 years' data to a limited pilot registry to answer some basic questions about presenting and diagnostic features and initial treatment practices at disease onset.