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| Predictors of Renal Outcome in Children with ANCA Associated Vasculitis: results of the ERKNet/ESPN/IPNA Survey |
MATKO MARLAIS
1
TANJA WLODKOWSKI
2
NIKOLETA PRINTZA
3
DOROTHEA KRONSTEINER
2
REGINA BRINSTER
2
MARINA AKSENOVA
4
ISA ASHOOR
5
ATIF AWAN
6
RAMNATH BALASUBRAMANIAN
7
BISWANATH BASU
8
ZIVILE BEKASSY
9
OLIVIA BOYER
10
EUGENE CHAN
11
CARLOS CHICA
12
DAGMAR CSAICSICH
13
STEPHANE DECRAMER
14
EISKE DORRESTEIJN
15
MAGDALENA DROZYNSKA-DUKLAS
16
LOAI EID
17
LAURA ESPINOSA
18
VERONICA FERRARIS
19
HANA FLÖGELOVA
20
JESSICA FORERO-DELGADILLO
21
VALENTINA GRACCHI
22
MERCEDES LóPEZ GONZáLEZ
23
MATTHIAS HANSEN
24
MOTOSHI HATTORI
25
NAKYSA HOOMAN
26
SWE LWIN HTUN
27
DMYTRO IVANOV
28
HEE GYUNG KANG
29
VASILIKI KARAVA
30
INA KAZYRA
31
AUDREY LAURENT
32
ADRIAN CATALIN LUNGU
33
STEPHEN MARKS
1
ANDREW MAXTED
34
ANNA MOCZULSKA
35
REBEKKA MUELLER
2
TATIANA NASTAUSHEVA
36
MATTIA PAROLIN
37
CARMINE PECORARO
38
ILIANA PRINCIPI
39
CHERYL SANCHEZ-KAZI
40
SEHA SAYGILI
41
RAPHAEL SCHILD
42
MOHAN SHENOY
43
RAJIV SINHA
44
ANA PAULA SPIZZIRRI
45
MARIA STACK
6
MARIA SZCZEPANSKA
46
ALEXEY TSYGIN
47
JULIA TZENG
48
VAIDOTAS URBONAS
49
TAO ZHANG
50
JAKUB ZIEG
51
FEDERICA ZOTTA
52
FRANZ SCHAEFER
2
MARINA VIVARELLI
52
KJELL TULLUS
1
1- GREAT ORMOND STREET HOSPITAL FOR CHILDREN NHS FOUNDATION TRUST
2- HEIDELBERG UNIVERSITY HOSPITAL
3- ARISTOTLE UNIVERSITY OF THESSALONIKI
4- PIROGOV RUSSIAN NATIONAL RESEARCH MEDICAL UNIVERSITY
5- CHILDRENS HOSPITAL, NEW ORLEANS
6- CHILDREN’S HEALTH IRELAND AT TEMPLE STREET, DUBLIN
7- EVELINA LONDON HOSPITAL FOR CHILDREN, LONDON
8- NIL RATAN SIRCAR MEDICAL COLLEGE, KOLKATA
9- UNIVERSITY HOSPITAL SUS LUND SWEDEN
10- HôPITAL NECKER-ENFANTS MALADES, AP-HP, PARIS
11- HONG KONG CHILDRENS HOSPITAL
12- HOSPITAL UNIVERSITARIO SAN JORGE - PEREIRA
13- MEDICAL UNIVERSITY OF VIENNA
14- CHU PURPAN, TOULOUSE
15- ERASMUSMC, ROTTERDAM
16- UNIVERSITY CLINICAL CENTRE, MEDICAL UNIVERSITY OF GDANSK
17- DUBAI HOSPITAL, DUBAI
18- HOSPITAL INFANTIL LA PAZ, MADRID
19- HOSPITAL ITALIANO DE BUENOS AIRES
20- FACULTY HOSPITAL, OLOMOUC, CZECH REPUBLIC
21- FUNDACION VALLE DEL LILI, COLOMBIA
22- BEATRIX CHILDRENS HOSPITAL - UMCG, GRONINGEN
23- VALL D´HEBRóN HOSPITAL, BARCELONA
24- KFH-NIERENZENTRUM FÜR KINDER UND JUGENDLICHE BEIM CLEMENTINE -KINDERHOSPITAL, FRANKFURT
25- TOKYO WOMENS MEDICAL UNIVERSITY, TOKYO
26- ALIASGHAR CLINICAL RESEARCH DEVELOPMENT CENTER, IRAN
27- MANDALAY CHILDREN HOSPITAL, MYANMAR
28- UKRAINE KIEV SHYPUK NATIONAL MEDICAL ACADEMY
29- SEOUL NATIONAL UNIVERSITY CHILDRENS HOSPITAL, SEOUL
30- 1ST DEPARTMENT OF PEDIATRICS, IPPOKRATIO GENERAL HOSPITAL, THESSALONIKI
31- BELARUS STATE MEDICAL UNIVERSITY, MINSK 2ND CHILDRENS HOSPITAL
32- HFME, LYON
33- FUNDENI CLINICAL INSTITUTE, BUCHAREST
34- NOTTINGHAM CHILDRENS HOSPITAL
35- JAGIELLONIAN UNIVERSITY MEDICAL COLLEGE, POLAND
36- VORONEZH STATE MEDICAL UNIVERSITY
37- AZIENDA OSPEDALIERA DI PADOVA
38- SANTOBONO CHILDRENS HOSPITAL
39- HOSPITAL H. NOTTI, MENDOZA
40- LOMA LINDA UNIVERSITY CHILDRENS HOSPITAL, CALIFORNIA
41- CERRAHPASA MEDICAL SCHOOL, ISTANBUL
42- UNIVERSITY CHILDRENS HOSPITAL HAMBURG
43- ROYAL MANCHESTER CHILDRENS HOSPITAL
44- INSTITUTE OF CHILD HEALTH, KOLKATA
45- HOSPITAL DE NIñOS SOR LUDOVICA, ARGENTINA
46- FACULTY OF MEDICAL SCIENCES IN ZABRZE, SUM IN KATOWICE
47- NATIONAL MEDICAL CENTRE FOR CHILDRENS HEALTH, RUSSIA
48- VALLEY CHILDRENS HEALTHCARE, MADERA
49- VILNIUS UNIVERSITY CLINIC FOR CHILDRENS DISEASES, VILNIUS
50- THE CHILDREN’S HOSPITAL OF FUDAN UNIVERSITY, SHANGHAI
51- MOTOL UNIVERSITY HOSPITAL
52- OSPEDALE PEDIATRICO BAMBINO GESù, ROME
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Introduction:
ANCA associated vasculitis (AAV) is a rare condition in children and data are limited on its treatment and renal outcomes. We aimed to determine clinical predictors of adverse renal outcome in children with AAV.
Material and methods:
Retrospective international survey conducted through professional paediatric nephrology organisations from December 2019 to March 2020. Paediatric nephrologists were asked to anonymously enter demographic and clinical data through an online form on all children presenting with AAV to their centre.
Results:
Adequate data were collected for 337 patients (from 41 countries, 72% female). Mean age at presentation 12.4 years, mean duration of follow-up 42 months. Mean peak serum creatinine at presentation was 377umol/L (4.26mg/dl) falling to a mean of 95umol/L (1.07mg/dl) at latest follow up in those not on KRT. 42% were classified as microscopic polyangiitis, 31% as granulomatosis with polyangiitis. 63% were from high income countries, 29% from upper-middle income countries (GNP 2019). There was an 5% mortality in this cohort, with 41% of patients requiring kidney replacement therapy (KRT) at any point. In multivariate mixed regression modelling, significant predictors of adverse renal outcome (requirement for KRT) included higher peak serum creatinine at presentation (p<0.001), ANCA-MPO positivity (p=0.002) and neurological involvement at presentation (p=0.02). Additionally, receiving plasma exchange as part of induction treatment was associated with a higher risk of having an abnormal creatinine at latest follow up in those not requiring KRT (p=0.0004).
Conclusions:
This large international cohort of children with AAV demonstrates the significant risk of chronic kidney disease and requirement for KRT in those presenting to paediatric nephrologists. Clinical predictors of adverse renal outcome at presentation are identified but further prospective research is required to determine the impact of treatment on clinical outcomes.
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