TY - JOUR
T1 - Lupus nephritis in children and adolescents
T2 - Results of the Italian Collaborative Study
AU - Ruggiero, Barbara
AU - Vivarelli, Marina
AU - Gianviti, Alessandra
AU - Benetti, Elisa
AU - Peruzzi, Licia
AU - Barbano, Giancarlo
AU - Corona, Fabrizia
AU - Ventura, Giovanna
AU - Pecoraro, Carmine
AU - Murer, Luisa
AU - Ghiggeri, Gian Marco
AU - Pennesi, Marco
AU - Edefonti, Alberto
AU - Coppo, Rosanna
AU - Emma, Francesco
PY - 2013/6
Y1 - 2013/6
N2 - BackgroundLupus 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.MethodsThe 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.ResultsAt 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.ConclusionsThe 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.
AB - BackgroundLupus 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.MethodsThe 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.ResultsAt 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.ConclusionsThe 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.
KW - ACR criteria
KW - Children
KW - Chronic renal failure
KW - Full house nephropathy
KW - Lupus nephritis
KW - Systemic lupus erythematosus
UR - http://www.scopus.com/inward/record.url?scp=84879861925&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84879861925&partnerID=8YFLogxK
U2 - 10.1093/ndt/gfs589
DO - 10.1093/ndt/gfs589
M3 - Article
C2 - 23345627
AN - SCOPUS:84879861925
VL - 28
SP - 1487
EP - 1496
JO - Nephrology Dialysis Transplantation
JF - Nephrology Dialysis Transplantation
SN - 0931-0509
IS - 6
ER -