Lupus nephritis in children and adolescents: results of the Italian Collaborative Study.
BACKGROUND
Lupus nephritis (LN) strongly affects the outcome in children with systemic lupus erythematosus (SLE). Many patients, however, have renal disease at onset, but lack a sufficient number of criteria to be diagnosed as SLE and develop delayed symptoms over time (d-SLE). Data on the clinical course, long-term outcome and predictors of disease progression in children with LN are scant.


METHODS
The Italian Collaborative Study included 161 paediatric patients with LN who were followed up for a mean of 96 months (range 6-296) in seven paediatric nephrology units. Cox-Mantel regression models were used to identify predictors of disease remission, relapse and progression.


RESULTS
At 1 year, the proportion of patients in remission was 83.2% (partial) and 53.5% (complete). Renal flares occurred in >50% of patients within 10 years. The intensity of induction treatment correlated significantly with the achievement of remission, while d-SLE, class IV LN and younger age were associated with poor response to treatment and/or with progression to chronic renal failure.


CONCLUSIONS
The current study provides outcome data on a large paediatric population with LN and underlines the importance of prescribing appropriate induction treatment to all children, regardless of the presence of enough SLE criteria, which may develop several years after the initial diagnosis.
Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
Barbara
B
Ruggiero
Marina
M
Vivarelli
Alessandra
A
Gianviti
Elisa
E
Benetti
Licia
L
Peruzzi
Giancarlo
G
Barbano
Fabrizia
F
Corona
Giovanna
G
Ventura
Carmine
C
Pecoraro
Luisa
L
Murer
Gian Marco
GM
Ghiggeri
Marco
M
Pennesi
Alberto
A
Edefonti
Rosanna
R
Coppo
Francesco
F
Emma
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000401:mortality* / Q000473:pathology; D008181:Lupus Nephritis / Q000150:complications / Q000401:mortality* / Q000628:therapy; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate
D007166:Immunosuppressive Agents
ACR criteria; children; chronic renal failure; full house nephropathy; lupus nephritis; systemic lupus erythematosus