Publication:
Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Issued Date

2017-07-01

Resource Type

Article

ISSN

23265205
23265191

DOI

10.1002/art.40112

Other identifier(s)

2-s2.0-85020421797

Rights

Mahidol University

Rights Holder(s)

SCOPUS

Bibliographic Citation

Arthritis and Rheumatology. Vol.69, No.7 (2017), 1470-1479

Suggested Citation

Kimberly A. Morishita, Lakshmi N. Moorthy, Joanna M. Lubieniecka, Marinka Twilt, Rae S.M. Yeung, Mary B. Toth, Susan Shenoi, Goran Ristic, Susan M. Nielsen, Raashid A. Luqmani, Suzanne C. Li, Tzielan Lee, Erica F. Lawson, Mikhail M. Kostik, Marisa Klein-Gitelman, Adam M. Huber, Aimee O. Hersh, Dirk Foell, Melissa E. Elder, Barbara A. Eberhard, Paul Dancey, Sirirat Charuvanij, Susanne M. Benseler, David A. Cabral Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. Arthritis and Rheumatology. Vol.69, No.7 (2017), 1470-1479. doi:10.1002/art.40112 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/42813

Research Projects

Organizational Units

Authors

Journal Issue

Thesis

Title

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Author(s)

Kimberly A. Morishita
 Lakshmi N. Moorthy
 Joanna M. Lubieniecka
 Marinka Twilt
 Rae S.M. Yeung
 Mary B. Toth
 Susan Shenoi
 Goran Ristic
 Susan M. Nielsen
 Raashid A. Luqmani
 Suzanne C. Li
 Tzielan Lee
 Erica F. Lawson
 Mikhail M. Kostik
 Marisa Klein-Gitelman
 Adam M. Huber
 Aimee O. Hersh
 Dirk Foell
 Melissa E. Elder
 Barbara A. Eberhard
 Paul Dancey
 Sirirat Charuvanij
 Susanne M. Benseler
 David A. Cabral

Other Contributor(s)

The University of British Columbia
 Rutgers Robert Wood Johnson Medical School
 Simon Fraser University
 University of Calgary
 Hospital for Sick Children University of Toronto
 Akron Children's Hospital
 Children's Hospital and Regional Medical Center
 Mother and Child Health Care Institute of Serbia
 Rigshospitalet
 University of Oxford
 The Joseph M. Sanzari Children's Hospital
 Stanford University School of Medicine
 University of California, San Francisco
 Saint Petersburg State Pediatric Medical University
 Ann & Robert H. Lurie Children's Hospital of Chicago
 IWK Health Centre
 University of Utah
 Universitätsklinikum Münster
 University of Florida
 Cohen Children's Medical Center of New York
 New Janeway Children s Health and Rehabilitation Centre
 Mahidol University

Abstract

© 2017, American College of Rheumatology Objective: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Methods: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4–6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. Results: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9–15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4–6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0–6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. Conclusion: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.

Keyword(s)

Immunology and Microbiology

Availability

URI

https://repository.li.mahidol.ac.th/handle/20.500.14594/42813

Collections

Scopus 2016-2017