PMID- 27813340
OWN - NLM
STAT- MEDLINE
DCOM- 20170913
LR  - 20191210
IS  - 2151-4658 (Electronic)
IS  - 2151-464X (Print)
IS  - 2151-464X (Linking)
VI  - 69
IP  - 9
DP  - 2017 Sep
TI  - Variation in the Treatment of Children Hospitalized With Antineutrophil 
      Cytoplasmic Antibody-Associated Vasculitis in the US.
PG  - 1377-1383
LID - 10.1002/acr.23142 [doi]
AB  - OBJECTIVE: There are few reports on the treatment of antineutrophil cytoplasmic 
      antibody-associated vasculitis (AAV) in children. This study characterizes the 
      use of cyclophosphamide, rituximab, and plasma exchange in children hospitalized 
      with AAV in the US. METHODS: We conducted a retrospective cohort study of 
      children hospitalized with AAV from 2004-2014 utilizing an administrative and 
      billing database from 47 tertiary care pediatric hospitals. All patients had an 
      International Classification of Diseases, Ninth Revision, Clinical Modification 
      discharge code of 446.4 and >/=1 charge for glucocorticoids. Treatment receipt was 
      determined using billing data. Mixed-effects logistic regression was used to 
      evaluate factors associated with the likelihood of receipt of each of the 3 
      treatments. RESULTS: During the 11-year study period there were 1,290 admissions 
      for 393 children. The median age at index admission was 14.6 years, and 61% were 
      female. Dialysis or mechanical ventilation was required by 16% and 17% of the 
      children, respectively. The median length of stay was 9 days. The percentages of 
      children receiving cyclophosphamide, rituximab, or both were 57%, 21%, and 10%, 
      respectively, and 22% received plasma exchange. Mechanical ventilation was 
      associated with the receipt of cyclophosphamide and plasma exchange, but not 
      rituximab. There was an increasing trend in the use of rituximab over time during 
      the study period (P < 0.05), and a decreasing trend in the use of 
      cyclophosphamide (P < 0.05). Treatment use varied significantly between 
      hospitals, especially for plasma exchange. CONCLUSION: The treatment of children 
      with severe AAV is shifting from cyclophosphamide to rituximab, and their need 
      for dialysis, mechanical ventilation, and prolonged hospitalization remains 
      common. Use of plasma exchange is highly variable.
CI  - (c) 2016, American College of Rheumatology.
FAU - James, Karen E
AU  - James KE
AUID- ORCID: 0000-0003-3783-7317
AD  - Children's Hospital of Philadelphia, Philadelphia, Pennsylvania (current address: 
      University of Utah, Salt Lake City).
FAU - Xiao, Rui
AU  - Xiao R
AD  - Children's Hospital of Philadelphia and Perelman School of Medicine, University 
      of Pennsylvania, Philadelphia.
FAU - Merkel, Peter A
AU  - Merkel PA
AD  - University of Pennsylvania, Philadelphia.
FAU - Weiss, Pamela F
AU  - Weiss PF
AD  - Children's Hospital of Philadelphia and Perelman School of Medicine, University 
      of Pennsylvania, Philadelphia.
LA  - eng
GR  - K23 AR059749/AR/NIAMS NIH HHS/United States
GR  - T32 HD064567/HD/NICHD NIH HHS/United States
PT  - Evaluation Study
PT  - Journal Article
DEP - 20170813
PL  - United States
TA  - Arthritis Care Res (Hoboken)
JT  - Arthritis care & research
JID - 101518086
RN  - 0 (Antirheumatic Agents)
RN  - 0 (Immunosuppressive Agents)
RN  - 4F4X42SYQ6 (Rituximab)
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
MH  - Adolescent
MH  - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*therapy
MH  - Antirheumatic Agents/*therapeutic use
MH  - Child
MH  - Child, Preschool
MH  - Combined Modality Therapy/trends
MH  - Cyclophosphamide/*therapeutic use
MH  - Dialysis/trends
MH  - Female
MH  - Hospitalization/*statistics & numerical data
MH  - Humans
MH  - Immunosuppressive Agents/therapeutic use
MH  - Length of Stay
MH  - Male
MH  - Plasma Exchange/methods/*statistics & numerical data
MH  - Respiration, Artificial/trends
MH  - Retrospective Studies
MH  - Rituximab/*therapeutic use
MH  - United States
PMC - PMC5415438
MID - NIHMS827897
EDAT- 2016/11/05 06:00
MHDA- 2017/09/14 06:00
PMCR- 2018/09/01
CRDT- 2016/11/05 06:00
PHST- 2016/04/12 00:00 [received]
PHST- 2016/10/19 00:00 [revised]
PHST- 2016/11/01 00:00 [accepted]
PHST- 2016/11/05 06:00 [pubmed]
PHST- 2017/09/14 06:00 [medline]
PHST- 2016/11/05 06:00 [entrez]
PHST- 2018/09/01 00:00 [pmc-release]
AID - 10.1002/acr.23142 [doi]
PST - ppublish
SO  - Arthritis Care Res (Hoboken). 2017 Sep;69(9):1377-1383. doi: 10.1002/acr.23142. 
      Epub 2017 Aug 13.