ESPN 54th Annual Meeting

ESPN 2022


 
Clinical factors at presentation influencing outcome of biopsy proven IgA Vasculitis Nephritis - a multicentre study of 1175 children
Katharina Rohner 1 Matko Marlais 2 Yo Han Ahn 3 Alaa Ali 4 Abrar Alsharief 5 Biswanath Basu 6 Anja Blejc 7 Evrim Kargin Cakici 8 Nur Canpolat 9 Eugen Yu-Hin Chan 10 Stephane Decramer 11 Filipa Durao 12 Anne M Durkan 13 Ali Duzova 14 Thomas Forbes 15 Junior Gahona Villegas 16 Nilufer Goknar 17 Valentina Gracchi 18 Tulin Gungor 8 Tomoko Horinouchi 19 Belde Kasap Demir 20 Yasuko Kobayashi 21 Mikael Koskela 22 Eda Didem Kurt-Sukur 14 Claudio La Scola 23 Dean Langan 24 Xiaozhong Li 25 Gabriele Malgieri 26 Antonio Mastrangelo 27 Jeesu Min 3 Malgorzata Mizerska-Wasiak 28 Nabila Mussaoui 11 Aytul Noyan 29 Matti Nuutinen 30 Takayuki Okamoto 32 Louise Oni 33 Michiel Oosterveld 34 Malgorzata Pańczyk-Tomaszewska 28 Gonul Parmaksiz 29 Andrea Pasini 23 Pornpimol Rianthavorn 35 Yunyan Shen 25 Rajiv Sinha 36 Rezan Topaloglu 14 Diletta Domenica Torres 37 Tomohiro Udagawa 38 Yok Chin Yap 39 Kjell Tullus 2

1- UNIVERSITY CHILDRENS HOSPITAL ZURICH
2- GREAT ORMOND STREET HOSPITAL LONDON
3- DEPARTMENT OF PEDIATRICS, SEOUL NATIONAL UNIVERSITY CHILDRENS HOSPITAL & SEOUL NATIONAL UNIVERSITY COLLEGE OF MEDICINE, SEOUL, REPUBLIC OF KOREA
4- GREAT NORTH CHILDRENS HOSPITAL NEWCASTLE, UK
5- QUEEN SILVIA CHILDRENS HOSPITAL, GÖTEBORG, SWEDEN
6- DIV. OF PEDIATRIC NEPHROLOGY, DEPARTMENT OF PEDIATRICS, NILRATAN SIRCAR MEDICAL COLLEGE & HOSPITAL, KOLKATA, INDIA
7- DEPARTMENT OF NEPHROLOGY, UNIVERSITY CHILDRENS HOSPITAL, UNIVERSITY MEDICAL CENTRE LJUBLJANA, SLOVENIA
8- DR SAMI ULUS MATERNITY AND CHILD HEALTH AND DISEASES TRAINING AND RESEARCH HOSPITAL, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ANKARA, TURKEY
9- CERRAHPASA MEDICAL SCHOOL, ISTANBUL UNIVERSITY-CERRAHPASA, TURKEY
10- PAEDIATRIC NEPHROLOGY CENTRE, HONG KONG CHILDRENS HOSPITAL, HONG KONG
11- HôPITAL DES ENFANTS, TOULOUSE, FRANCE
12- PEDIATRIC NEPHROLOGY AND KIDNEY TRANSPLANTATION UNIT, DEPARTMENT OF PEDIATRICS, HOSPITAL SANTA MARIA, CENTRO HOSPITALAR UNIVERSITáRIO LISBOA NORTE, LISBON, PORTUGAL.
13- DEPT OF NEPHROLOGY, THE CHILDREN’S HOSPITAL AT WESTMEAD, SYDNEY, AUSTRALIA
14- HACETTEPE UNIVERSITY SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRICS, DIVISION OF NEPHROLOGY, ANKARA, TURKEY
15- DEPARTMENT OF NEPHROLOGY, ROYAL CHILDREN’S HOSPITAL, VICTORIA, AUSTRALIA
16- BACA ORTIZ PEDIATRIC HOSPITAL (QUITO-ECUADOR), PONTIFICAL CATHOLIC UNIVERSITY OF ECUADOR
17- ISTANBUL MEDENIYET UNIVERSITY, TURKEY
18- BEATRIX CHILDREN’S HOSPITAL, UNIVERSITY MEDICAL CENTER GRONINGEN, UNIVERSITY OF GRONINGEN, GRONINGEN, THE NETHERLANDS
19- DEPARTMENT OF PEDIATRICS, KOBE UNIVERSITY GRADUATE SCHOOL OF MEDICINE, KOBE, JAPAN
20- İZMIR KATIP ÇELEBI UNIVERSITY, DEPARTMENT OF PEDIATRICS, DIVISION OF PEDIATRIC NEPHROLOGY AND RHEUMATOLOGY, TURKEY
21- GUNMA UNIVERSITY GRADUATE SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRIC, GUNMA, JAPAN
22- DEPARTMENT OF PEDIATRIC NEPHROLOGY AND TRANSPLANTATION, NEW CHILDRENS HOSPITAL, UNIVERSITY OF HELSINKI AND HELSINKI UNIVERSITY HOSPITAL, HELSINKI, FINLAND.
23- IRCCS AZIENDA OSPEDALIERO-UNIVERSIATRIA DI BOLOGNA, ITALY
24- UNIVERSITY COLLEGE LONDON, LONDON, UK
25- THE CHILDRENS HOSPITAL OF SOOCHOW, CHINA
26- DIVISION OF NEPHROLOGY,DIALYSIS AND TRANSPLANT, AORN SANTOBONO PAUSILIPON, NAPOLI, ITALY
27- NEFROLOGIA, DIALISI E TRAPIANTO PEDIATRICO (NDTP), FONDAZIONE IRCCS CA GRANDA OSPEDALE MAGGIORE POLICLINICO, MILANO, ITALY
28- DEPARTMENT OF PEDIATRICS AND NEPHROLOGY, MEDICAL UNIVERSITY OF WARSAW, POLAND
29- BASKENT UNIVERSITY ADANA DR. TURGUT NOYAN TRAINING AND RESEARCH CENTER, ANDANA, TURKEY
30- CLINIC FOR CHILDREN AND ADOLESCENTS, OULU UNIVERSITY HOSPITAL, OULU, FINLAND
31- ROYAL BELFAST HOSPITAL FOR SICK CHILDREN, BELFAST, IRELAND
32- DEPARTMENT OF PEDIATRICS, HOKKAIDO UNIVERSITY GRADUATE SCHOOL OF MEDICINE, SAPPORO, JAPAN
33- DEPARTMENT OF WOMENS AND CHILDRENS HEALTH, ALDER HEY CHILDRENS NHS FOUNDATION TRUST HOSPITAL AND UNIVERSITY OF LIVERPOOL, UK
34- EMMA CHILDREN’S HOSPITAL, AMSTERDAM UNIVERSITY MEDICAL CENTER, AMSTERDAM, NETHERLANDS
35- DEPARTMENT OF PEDIATRICS, CHULALONGKORN UNIVERSITY, BANGKOK, THAILAND
36- DIVISION OF PEDIATRIC NEPHROLOGY, INSTITUTE OF CHILD HEALTH, KOLKATA, INDIA.
37- PEDIATRIC NEPHROLOGY AND DIALYSIS UNIT, PEDIATRIC HOSPITAL GIOVANNI XXIII, BARI, ITALY
38- TOKYO MEDICAL AND DENTAL UNIVERSITY, TOKYO, JAPAN
39- DEPARTMENT OF PAEDIATRIC, HOSPITAL TUNKU AZIZAH, WOMEN AND CHILDREN HOSPITAL KUALA LUMPUR, MALAYSIA
 
Introduction:

 

IgA Vasculitis Nephritis (IgAVN) is in a large majority of cases self-limiting but a small proportion of affected children develop marked proteinuria, worsening kidney function and even kidney failure. The aim of our study was to define the clinical parameters at onset that have an effect on outcome in a large cohort of children with kidney biopsy-proven IgAVN.

Material and methods:

 

Data were collected through a retrospective international survey from December 2020 to August 2021. Anonymised demographic and clinical data (including renal outcome data) were collected for children (0-18 years of age) with typical symptoms of IgA-Vasculitis, biopsy-proven IgAVN and a follow up of at least 12 months.

Results:

Data from 1175 patients were collected from 42 international paediatric nephrology centres. Median age at kidney biopsy was 8.3 years, 43 % female, median duration of follow up 3.7 years. At time of biopsy 37 patients (3.5 %) had an estimated glomerular filtration rate (eGFR) below 30 ml/min/1.73m2 and 2.6 % needed kidney replacement therapy. 92.5% of the patients had proteinuria, 50% within nephrotic range. With increasing age children had lower eGFR but also lower proteinuria at presentation (p=0.000 and p=0.008).

 

54.1 % had normalised their kidney function, 40.4 % had CKD Stage 1 and 2, 5.5 % CKD 3-5 at last follow up. 76.6 % had normalised urinary protein excretion, 1.5% continued to have nephrotic range proteinuria. Lower eGFR (p=0.000), hypoalbuminemia (p=0.027) and nephrotic syndrome (p=0.002) at onset were predictors of impaired eGFR at last follow up.

Conclusions:

This cohort is the largest study to date of children with biopsy proven IgAVN and confirms the overall favourable outcome in the medium term. Nephrotic syndrome and impaired kidney function at onset increased the risk of developing CKD in the medium term.