PMID- 33675161
OWN - NLM
STAT- MEDLINE
DCOM- 20220831
LR  - 20220916
IS  - 2151-4658 (Electronic)
IS  - 2151-464X (Linking)
VI  - 74
IP  - 9
DP  - 2022 Sep
TI  - Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic 
      Antibody-Associated Vasculitis.
PG  - 1550-1558
LID - 10.1002/acr.24590 [doi]
AB  - OBJECTIVE: There is no standardized approach to the treatment of pediatric 
      antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Because of the 
      rarity of pediatric AAV, randomized trials have not been feasible. The present 
      study of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) was 
      undertaken to establish consensus treatment plans (CTPs) for severe pediatric AAV 
      to enable the future study of comparative effectiveness and safety. METHODS: A 
      workgroup of CARRA members (rheumatologists and nephrologists) formed the AAV 
      Workgroup. This group performed a literature review on existing evidence-based 
      treatments and guidelines for the management of AAV. They determined that the 
      target population for CTP development was patients <18 years of age with 
      new-onset granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or 
      renal-limited AAV (eosinophilic GPA was excluded), with presentation confined to 
      those with severe disease (i.e., organ- or life-threatening). Face-to-face 
      consensus conferences employed nominal group techniques to identify treatment 
      strategies for remission induction and remission maintenance, data elements to be 
      systematically collected, and outcomes to be measured over time. RESULTS: The 
      pediatric AAV Workgroup developed 2 CTPs for each of the remission induction and 
      remission maintenance of severe AAV. A glucocorticoid-weaning regimen for 
      induction and maintenance, a core data set, and outcome measures were also 
      defined. A random sample of CARRA membership voted acceptance of the CTPs for 
      remission induction and remission maintenance, with a 94% (75 of 80) and 98% (78 
      of 80) approval rate, respectively. CONCLUSION: Consensus methodology established 
      standardized CTPs for treating severe pediatric AAV. These CTPs were in principle 
      accepted by CARRA-wide membership for the evaluation of pragmatic comparative 
      effectiveness in a long-term registry.
CI  - (c) 2021 American College of Rheumatology.
FAU - Morishita, Kimberly A
AU  - Morishita KA
AD  - British Columbia's Children's Hospital and the University of British Columbia, 
      Vancouver, British Columbia, Canada.
FAU - Wagner-Weiner, Linda
AU  - Wagner-Weiner L
AD  - University of Chicago Medical Center, Chicago, Illinois.
FAU - Yen, Eric Y
AU  - Yen EY
AUID- ORCID: 0000-0002-7632-6790
AD  - University of California, Los Angeles.
FAU - Sivaraman, Vidya
AU  - Sivaraman V
AD  - Nationwide Children's Hospital and The Ohio State University, Columbus.
FAU - James, Karen E
AU  - James KE
AD  - University of Utah, Salt Lake City.
FAU - Gerstbacher, Dana
AU  - Gerstbacher D
AD  - Stanford University, Stanford, California.
FAU - Szymanski, Ann M
AU  - Szymanski AM
AD  - Johns Hopkins All Children's Hospital, Saint Petersburg, Florida.
FAU - O'Neil, Kathleen M
AU  - O'Neil KM
AD  - Riley Hospital for Children at Indiana University Health, Indianapolis.
FAU - Cabral, David A
AU  - Cabral DA
AD  - British Columbia's Children's Hospital and the University of British Columbia, 
      Vancouver, British Columbia, Canada.
CN  - Childhood Arthritis and Rheumatology Research Alliance (CARRA) Antineutrophil 
      Cytoplasmic Antibody-Associated Vasculitis Workgroup
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20220601
PL  - United States
TA  - Arthritis Care Res (Hoboken)
JT  - Arthritis care & research
JID - 101518086
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
SB  - IM
MH  - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis/drug 
      therapy
MH  - Antibodies, Antineutrophil Cytoplasmic
MH  - *Arthritis, Juvenile
MH  - Child
MH  - Humans
MH  - *Microscopic Polyangiitis
MH  - *Rheumatology
EDAT- 2021/03/07 06:00
MHDA- 2022/09/01 06:00
CRDT- 2021/03/06 05:46
PHST- 2021/02/17 00:00 [revised]
PHST- 2020/10/19 00:00 [received]
PHST- 2021/03/02 00:00 [accepted]
PHST- 2021/03/07 06:00 [pubmed]
PHST- 2022/09/01 06:00 [medline]
PHST- 2021/03/06 05:46 [entrez]
AID - 10.1002/acr.24590 [doi]
PST - ppublish
SO  - Arthritis Care Res (Hoboken). 2022 Sep;74(9):1550-1558. doi: 10.1002/acr.24590. 
      Epub 2022 Jun 1.