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Multicenter Study
. 2017 Aug 7;15(1):61.
doi: 10.1186/s12969-017-0191-z.

Clinical practice variation and need for pediatric-specific treatment guidelines among rheumatologists caring for children with ANCA-associated vasculitis: an international clinician survey

Clara Westwell-Roper  1 , Joanna M Lubieniecka  2 , Kelly L Brown  1 , Kimberly A Morishita  1 , Cherry Mammen  1 , Linda Wagner-Weiner  3 , Eric Yen  4 , Suzanne C Li  5 , Kathleen M O'Neil  6 , Sivia K Lapidus  7 , Paul Brogan  8 , Rolando Cimaz  9 , David A Cabral  10 ; for ARChiVe Investigators Network within the PedVas initiative
Affiliations
Multicenter Study

Clinical practice variation and need for pediatric-specific treatment guidelines among rheumatologists caring for children with ANCA-associated vasculitis: an international clinician survey

Clara Westwell-Roper et al. Pediatr Rheumatol Online J. .

Abstract

Background: Because pediatric antineutrophil cytoplasmic antibody-associated vasculitis is rare, management generally relies on adult data. We assessed treatment practices, uptake of existing clinical assessment tools, and interest in pediatric treatment protocols among rheumatologists caring for children with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

Methods: A needs-assessment survey developed by an international working group of pediatric rheumatologists and two nephrologists was circulated internationally. Data were summarized with descriptive statistics. Pearson's chi-square tests were used in inferential univariate analyses.

Results: The 209 respondents from 36 countries had collectively seen ~1600 children with GPA/MPA; 144 had seen more than two in the preceding 5 years. Standardized and validated clinical assessment tools to score disease severity, activity, and damage were used by 59, 63, and 36%, respectively; barriers to use included lack of knowledge and limited perceived utility. Therapy varied significantly: use of rituximab rather than cyclophosphamide was more common among respondents from the USA (OR = 2.7 [1.3-5.5], p = 0.0190, n = 139), those with >5 years of independent practice experience (OR = 3.8 [1.3-12.5], p = 0.0279, n = 137), and those who had seen >10 children with GPA/MPA in their careers (OR = 4.39 [2.1-9.1], p = 0.0011, n = 133). Respondents who had treated >10 patients were also more likely to continue maintenance therapy for at least 24 months (OR = 3.0 [1.4-6.4], p = 0.0161, n = 127). Ninety six percent of respondents believed in a need for pediatric-specific treatment guidelines; 46% supported adaptation of adult guidelines while 69% favoured guidelines providing a limited range of treatment options to allow comparison of effectiveness through a registry.

Conclusions: These data provide a rationale for developing pediatric-specific consensus treatment guidelines for GPA/MPA. While pediatric rheumatologist uptake of existing clinical tools has been limited, guideline uptake may be enhanced if outcomes of consensus-derived treatment options are evaluated within the framework of an international registry.

Keywords: Anti-neutrophil cytoplasmic antibody-associated vasculitis; Clinical practice guidelines; Disease classification; Granulomatosis with polyangiitis; Microscopic polyangiitis; Outcome assessment; Pediatric rheumatology; Physician practice patterns; Vasculitis treatment.

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Conflict of interest statement

Ethics approval and consent to participate

The survey was conducted as a quality assurance initiative for PedVas, which was approved by the Children’s and Women’s Research Ethics Board of the University of British Columbia (H12-00894). Under Article 2.5 of the Tri Council Policy Statement, quality assurance/improvement activities are not subject to further institutional review.

Consent for publication

Not applicable

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Typical dosing regimens prescribed for cyclophosphamide (CYC) and rituximab (RTX) induction therapy. Data represent number of respondents out of n = 140 for both agents. aEUVAS protocol
See this image and copyright information in PMC

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