Establishment of a pilot pediatric registry for chronic vasculitis is both essential and feasible: a Childhood Arthritis and Rheumatology Alliance (CARRA) survey

J Rheumatol. 2007 Jan;34(1):224-6. Epub 2006 Nov 15.

Abstract

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.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Canada / epidemiology
  • Child
  • Child, Preschool
  • Chronic Disease
  • Feasibility Studies
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / epidemiology
  • Granulomatosis with Polyangiitis / therapy
  • Health Surveys*
  • Humans
  • Needs Assessment
  • Pilot Projects
  • Polyarteritis Nodosa / diagnosis
  • Polyarteritis Nodosa / epidemiology
  • Polyarteritis Nodosa / therapy
  • Prospective Studies
  • Registries*
  • Rheumatology / statistics & numerical data
  • Takayasu Arteritis / diagnosis
  • Takayasu Arteritis / epidemiology
  • Takayasu Arteritis / therapy
  • United States / epidemiology
  • Vasculitis / diagnosis
  • Vasculitis / epidemiology*
  • Vasculitis / therapy
  • Workforce