Literature DB >> 17117484

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

Nicholas M R Wilkinson1, Jacqueline Page, América G Uribe, Victor Espinosa, David A Cabral.   

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.

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Year:  2006        PMID: 17117484

Source DB:  PubMed          Journal:  J Rheumatol        ISSN: 0315-162X            Impact factor:   4.666


  10 in total

Review 1.  Granulomatosis with polyangiitis in childhood.

Authors:  Marinka Twilt; Susanne Benseler; David Cabral
Journal:  Curr Rheumatol Rep       Date:  2012-04       Impact factor: 4.592

2.  [EULAR/PreS consensus criteria 2006 for the classification of childhood vasculitides].

Authors:  H-I Huppertz
Journal:  Z Rheumatol       Date:  2009-11       Impact factor: 1.372

3.  Physician assessment of disease activity in JIA subtypes. Analysis of data extracted from electronic medical records.

Authors:  Michael L Miller; Jason Ruprecht; Deli Wang; Ying Zhou; George Lales; Sean McKenna; Marisa Klein-Gitelman
Journal:  Pediatr Rheumatol Online J       Date:  2011-04-14       Impact factor: 3.054

4.  Churg-strauss syndrome without respiratory symptoms in a child.

Authors:  R B Basak; H Narchi; M Bakir; S Joshi; W Conca
Journal:  Indian J Dermatol       Date:  2011-01       Impact factor: 1.494

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

Authors:  Clara Westwell-Roper; Joanna M Lubieniecka; Kelly L Brown; Kimberly A Morishita; Cherry Mammen; Linda Wagner-Weiner; Eric Yen; Suzanne C Li; Kathleen M O'Neil; Sivia K Lapidus; Paul Brogan; Rolando Cimaz; David A Cabral
Journal:  Pediatr Rheumatol Online J       Date:  2017-08-07       Impact factor: 3.054

6.  Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey.

Authors:  Angela S Quan; Jürgen Brunner; Benjamin Rose; Martin Smitka; Gabriele Hahn; Clare E Pain; Renate Häfner; Fabian Speth; Lucia Gerstl; Christian M Hedrich
Journal:  Front Pediatr       Date:  2021-03-26       Impact factor: 3.418

7.  A pseudoleukemic blood differentiation in a 13-year-old child: an extraordinary presentation of Churg-Strauss syndrome.

Authors:  E R Mutsaers; R Witteveen; W van den Bosch-Ruis; T W Kuijpers; M A van Houten; J M van den Berg
Journal:  Clin Rheumatol       Date:  2009-09-09       Impact factor: 2.980

Review 8.  Pediatric Vasculitis.

Authors:  Davinder Singh-Grewal; Anne Maria Durkan
Journal:  Indian J Pediatr       Date:  2015-09-14       Impact factor: 5.319

9.  Upper respiratory system involvement as the only manifestation of granulomatosis with polyangiitis in a child with marfan phenotype.

Authors:  Reza Amin; Soheila Alyasin; Tooba Momen; Maryam Khoshkhouy; Sepideh Darougar
Journal:  Tanaffos       Date:  2013

10.  The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age.

Authors:  Clara Preto; Armandina Silva; Sandra Alves; Margarida Guedes; Paula Matos; Conceição Mota; Paula Rocha; Paula C Fernandes
Journal:  Case Rep Pediatr       Date:  2017-12-19
  10 in total

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