ESPN 50th Annual Meeting

ESPN 2017


 
BETTER OUTCOME OF HENOCH-SCHĂ–NLEIN PURPURA NEPHRITIS IN CHILDREN: ANALYSIS FROM THE EUROPEAN HSPN REGISTRY
LICIA PERUZZI 1 FLAVIO TARIZZO 1 BRUNO GIANOGLIO 1 ROBERTA CAMILLA 1 FRANCESCA MATTOZZI 1 CARMINE PECORARO 2 ANNALISA ALESSANDRELLA 2 LUISA MURER 3 ELISA BENETTI 3 GEORGES DESCHENES 4 MARCO MATERASSI 5 PAMELA GALLO 5 FRANCESCA LUGANI 6 GIANMARCO GHIGGERI 6 BRANKICA SPASOJEVIC DIMITRIJEVA 7 AUGUSTINA JANKAUSKIENE 8 MALGORZATA MIZERSKA-WASIAK 9 ADRIAN LUNGU 10 REZAN TOPALOGLU 11 TOGAY YILMAZ 11 ROSANNA COPPO 1

1- REGINA MARGHERITA HOSPITAL, NEPHROLOGY DIALYSIS TRANSPLANTATION, TURIN, ITALY
2- SANTOBONO HOSPITAL, NEPHROLOGY AND DIALYSIS, NAPLES, ITALY
3- PADUA UNIVERSITY HOSPITAL, NEPHROLOGY DIALYSIS AND TRANSPLANTATION, PADUA, ITALY
4- ROBERT-DEBRÉ HOSPITAL, PEDIATRIC NEPHROLOGY, PARIS, FRANCE
5- MEYER HOSPITAL, NEPHROLOGY DIALYSIS, FLORENCE, ITALY
6- GASLINI HOSPITAL, NEPHROLOGY DIALYSIS TRANSPLANTATION, GENOA, ITALY
7- MOTHER AND CHILD UNIVERSITY HOSPITAL, NEPHROLOGY AND DIALYSIS, BELGRADE, SERBIA
8- UNIVERSITY HOSPITAL, NEPHROLOGY AND DIALYSIS, VILNIUS, LITHUANIA
9- MEDICAL UNIVERSITY OF WARSAW, PEDIATRICS AND NEPHROLOGY, WARSAW, POLAND
10- FUNDENI CLINICAL INSTITUTE, PEDIATRIC NEPHROLOGY, BUCHAREST, ROMANIA
11- HACETTEPE UNIVERSITY HOSPITAL, PEDIATRIC NEPHROLOGY, ANKARA, TURKEY
 
Introduction:

Henoch-Schönlein Purpura Nephritis (HSPN) progression to ESRD reached 20% in old series. In 2015 ESPN supported the European Registry on biopsy proven HSPN in children.

Material and methods:

Aim of the retrospective Registry is the identification of progression risk factors in children diagnosed and  followed since 1995.

Results:

203 children from 11 European centers were enrolled (58% M,81% Caucasians). Median age at onset was  8.17 (IQR 6-10.3); dominant extra-renal sign was purpura(86%), associated to abdominal pain(30%) or arthralgias(21%).
Renal involvement appeared 13 days median after purpura (IQR 0-45). Renal biopsy was performed 37 days median after signs of nephritis (IQR 16-95).
At time of biopsy 61% had received steroids, 5% steroids and immunosuppressors, 15% RAS–blockers, 32% no-therapy.
Renal histology was supplied by individual Centers according to ISKDC and Oxford MEST : ISKDC I:10.4%, II:36.9%, IIIa:33.2%; IIIb:14.7%, IVa:1.5%, IVb:2.9%, Vb:0.4% and Oxford MEST scores  M: M0 44.3%, M1 55.7%; E: E0 58.9%, E1 41.1%; S: S0 78.9%, S1 21.1%; T: T0 89.1%, T1 10.3%, T2 0.9%.
Median follow-up 5.75 years (IQR2.9- 8.3). 135/203 (66.5%) reached remission of hematuria at 7.39 months median (IQR 3.27-16.97) and 145/203 (71.4%) of proteinuria at 8.07 months median (IQR 4.83-15.89). One patient (1/203; 0.5%) reached ESRD 15 months after disease onset, none died. 
In this  cohort histology was not predictive for hematuria  or proteinuria remission according to ISKDC classification (ISKDC I+II vs ISKDC III to V; p=ns) or MEST (M0 vs M1, E0 vs E1, S0 vs S1, T0 vs T1-2; all p=ns), however in those not reaching remission a significant proportion of M1 and S1 was observed(Chi-square p=0.0006; Fisher p=0.0002).
 

Conclusions:

The analysis on 203 children enrolled so far indicates a substantially improved prognosis. No renal  histology was predictive of remission. Analysis of treatments modalities is expected to enlight the possible role in achieving better outcome.