PMID- 34550486
OWN - NLM
STAT- MEDLINE
DCOM- 20220802
LR  - 20220802
IS  - 1591-9528 (Electronic)
IS  - 1591-8890 (Linking)
VI  - 22
IP  - 3
DP  - 2022 Aug
TI  - Clinical features and outcomes of anti-neutrophil cytoplasmic 
      autoantibody-associated vasculitis in Chinese childhood-onset patients.
PG  - 447-453
LID - 10.1007/s10238-021-00762-4 [doi]
AB  - Data on anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are 
      limited in children. This study is to determine the clinical features and 
      outcomes of childhood-onset AAV. A retrospective study was performed on patients 
      who were diagnosed with AAV before 18 years old in Xiangya Hospital. Their 
      medical records were analyzed by retrospective review. Sixteen patients were 
      diagnosed with AAV before 18 years old in the past 9 years, with an average age 
      of 13.3 +/- 3.3 years and 13 of them were female. There were 15 patients with 
      microscopic polyangiitis (MPA) and 1 with Wegener's granulomatosis. The interval 
      between onset of disease and diagnosis of AAV was 2 (1.5-3) months. Most patients 
      (15/16, 93.8%) had multi-organ involvement, and all patients had renal 
      involvement with 7 (43.8%) patients requiring dialysis at presentation. Eleven 
      patients underwent a renal biopsy, of which mixed class and sclerotic class were 
      the most two common histological types. All patients received immunosuppressive 
      therapy for induction therapy including intravenous administrations of 
      methylprednisolone (MP) pulse therapy for 8 patients. 8 patients (50%) achieved 
      remission after induction therapy. After a median follow-up of 
      46.3 +/- 36.1 months, nine (56.3%) patients progressed to end-stage renal disease 
      (ESRD) and 5 (31.3%) patients died. Childhood-onset AAV showed similar clinical 
      and pathological features compared to those of adults, except that it usually 
      occurs in girls. The most commonly involved organ was the kidney, and it had a 
      high risk of progression to ESRD. Early diagnosis and initiation of appropriate 
      immunomodulatory therapy would be important to improve outcomes.
CI  - (c) 2021. The Author(s), under exclusive licence to Springer Nature Switzerland AG.
FAU - Meng, Ting
AU  - Meng T
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Shen, Chanjuan
AU  - Shen C
AD  - Department of Hematology, The Affiliated Zhuzhou Hospital Xiangya Medical 
      College, Central South University, Zhuzhou, Hunan Province, China.
FAU - Tang, Rong
AU  - Tang R
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Lin, Wei
AU  - Lin W
AD  - Department of Pathology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Ooi, Joshua D
AU  - Ooi JD
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
AD  - Centre for Inflammatory Diseases, Department of Medicine, Monash University, 
      Monash Medical Centre, Clayton, VIC, Australia.
FAU - Eggenhuizen, Peter J
AU  - Eggenhuizen PJ
AD  - Centre for Inflammatory Diseases, Department of Medicine, Monash University, 
      Monash Medical Centre, Clayton, VIC, Australia.
FAU - Zhou, Ya-Ou
AU  - Zhou YO
AUID- ORCID: 0000-0001-8736-0989
AD  - Department of Rheumatology and Immunology, Xiangya Hospital Central South 
      University, 87 Xiangya Road, Changsha, 410008, Hunan, People's Republic of China. 
      zhouyaou@csu.edu.cn.
FAU - Chen, Jinbiao
AU  - Chen J
AD  - Department of Medical Records and Information, Xiangya Hospital, Central South 
      University, Changsha, Hunan Province, China.
FAU - He, Fang
AU  - He F
AD  - Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
AD  - Hunan Intellectual and Developmental Disabilities Research Center, Changsha, 
      China.
FAU - Xiao, Zhou
AU  - Xiao Z
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Ao, Xiang
AU  - Ao X
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Peng, Weisheng
AU  - Peng W
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Nie, Wannian
AU  - Nie W
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Zhou, Qiaoling
AU  - Zhou Q
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Xiao, Ping
AU  - Xiao P
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Zhong, Yong
AU  - Zhong Y
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
FAU - Xiao, Xiangcheng
AU  - Xiao X
AD  - Department of Nephrology, Xiangya Hospital, Central South University, Changsha, 
      Hunan Province, China.
LA  - eng
GR  - 81800641/national natural science foundation of china/
GR  - 2020YFC2005000/national key r&d program of china/
GR  - 2018WK2060/key research and development program of hunan province/
GR  - 2020WK2008/key research and development program of hunan province/
GR  - 2020RC5002/science and technology innovation program of hunan province/
GR  - 2020JJ6109/natural science foundation of hunan province/
GR  - 2019JJ40515/natural science foundation of hunan province/
GR  - 18020010780/chinese society of nephrology/
PT  - Journal Article
DEP - 20210922
PL  - Italy
TA  - Clin Exp Med
JT  - Clinical and experimental medicine
JID - 100973405
RN  - 0 (Autoantibodies)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis/drug 
      therapy
MH  - Autoantibodies
MH  - Child
MH  - China
MH  - Female
MH  - Humans
MH  - *Kidney Failure, Chronic/complications
MH  - Male
MH  - Renal Dialysis
MH  - Retrospective Studies
OTO - NOTNLM
OT  - Anti-neutrophil cytoplasmic antibody-associated vasculitis
OT  - Childhood-onset
OT  - Clinical features
OT  - Outcomes
OT  - Renal involvement
EDAT- 2021/09/23 06:00
MHDA- 2022/08/03 06:00
CRDT- 2021/09/22 12:29
PHST- 2021/07/22 00:00 [received]
PHST- 2021/09/07 00:00 [accepted]
PHST- 2021/09/23 06:00 [pubmed]
PHST- 2022/08/03 06:00 [medline]
PHST- 2021/09/22 12:29 [entrez]
AID - 10.1007/s10238-021-00762-4 [pii]
AID - 10.1007/s10238-021-00762-4 [doi]
PST - ppublish
SO  - Clin Exp Med. 2022 Aug;22(3):447-453. doi: 10.1007/s10238-021-00762-4. Epub 2021 
      Sep 22.