PMID- 26200622
OWN - NLM
STAT- MEDLINE
DCOM- 20151020
LR  - 20221005
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 94
IP  - 29
DP  - 2015 Jul
TI  - Coexistence of Anti-Glomerular Basement Membrane Antibodies and Anti-Neutrophil 
      Cytoplasmic Antibodies in a Child With Human Leukocyte Antigen Susceptibility and 
      Detailed Antibody Description: A Case Report.
PG  - e1179
LID - 10.1097/MD.0000000000001179 [doi]
LID - e1179
AB  - Anti-glomerular basement membrane (anti-GBM) disease and anti-neutrophil 
      cytoplasmic antibody (ANCA)-associated vasculitis both could cause rapidly 
      progressive glomerulonephritis. The coexistence of ANCAs and anti-GBM antibodies 
      was known as "double positive," which was extremely rare in children. We report a 
      pediatric case with coexistence of ANCAs and anti-GBM antibodies. A 6-year-old 
      girl presented with acute renal failure, hematuria, proteinuria, and oliguria. 
      She was double positive of ANCAs specific to myeloperoxidase, and anti-GBM 
      antibodies. Kidney biopsy confirmed linear immunoglobulin (Ig)G deposit along GBM 
      and 100% of crescent formation in glomeruli; among them 83.3% were cellular 
      crescents. Human leukocyte antigen (HLA) gene typing showed DRB1*1501, an allele 
      strongly associated with anti-GBM disease, and DRB1*0405, an independent risk 
      factor for renal failure in patients with ANCA-associated vasculitis. The titer 
      of anti-GBM antibodies was 1:800, and the predominant IgG subclass was IgG1, 
      which was closely related with severe kidney injury and worse outcome. The target 
      antigen of anti-GBM antibodies was restricted on the noncollagen domain 1 of the 
      alpha3 chain of type IV collagen (alpha3[IV]NC1), with recognitions to both epitopes, EA 
      (alpha317-31) and EB (alpha3127-141). This is the first reported pediatric case with 
      coexistence of ANCAs and anti-GBM antibodies, in which the HLA typing and 
      immunologic characters of autoantibodies were identified. The findings on this 
      early-onset patient are meaningful for understanding the mechanisms of both 
      anti-GBM disease and ANCA-associated vasculitis.
FAU - Xie, Li-Jun
AU  - Xie LJ
AD  - From the Renal Division, Department of Medicine, Peking University First 
      Hospital; Institute of Nephrology, Peking University; Key Laboratory of Renal 
      Disease, Ministry of Health of China; Key Laboratory of CKD Prevention and 
      Treatment, Ministry of Education of China (L-jX, ZC, X-yJ, M-hZ); Department of 
      Nephrology, Beijing Children's Hospital affiliated to Capital Medical University 
      (ZC, X-rL); Renal Division, Department of Medicine, First Affiliated Hospital of 
      Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, 
      China (L-jX); and Peking-Tsinghua Center for Life Sciences, Beijing, PR China 
      (M-hZ).
FAU - Cui, Zhao
AU  - Cui Z
FAU - Jia, Xiao-Yu
AU  - Jia XY
FAU - Chen, Zhi
AU  - Chen Z
FAU - Liu, Xiao-Rong
AU  - Liu XR
FAU - Zhao, Ming-Hui
AU  - Zhao MH
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
RN  - 0 (Autoantibodies)
RN  - 0 (HLA Antigens)
RN  - 0 (Immunoglobulin G)
RN  - 0 (antiglomerular basement membrane antibody)
SB  - IM
MH  - Antibodies, Antineutrophil Cytoplasmic/blood/*immunology
MH  - Autoantibodies/blood/*immunology
MH  - Child
MH  - Female
MH  - HLA Antigens/genetics
MH  - Humans
MH  - Immunoglobulin G/blood
MH  - Renal Insufficiency/*immunology
PMC - PMC4603008
COIS- The authors declare no conflict of interest.
EDAT- 2015/07/23 06:00
MHDA- 2015/10/21 06:00
PMCR- 2015/07/24
CRDT- 2015/07/23 06:00
PHST- 2015/07/23 06:00 [entrez]
PHST- 2015/07/23 06:00 [pubmed]
PHST- 2015/10/21 06:00 [medline]
PHST- 2015/07/24 00:00 [pmc-release]
AID - 00005792-201507040-00014 [pii]
AID - 10.1097/MD.0000000000001179 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2015 Jul;94(29):e1179. doi: 10.1097/MD.0000000000001179.