Lupus nephritis in children and adolescents: Results of the Italian Collaborative Study

Barbara Ruggiero, Marina Vivarelli, Alessandra Gianviti, Elisa Benetti, Licia Peruzzi, Giancarlo Barbano, Fabrizia Corona, Giovanna Ventura, Carmine Pecoraro, Luisa Murer, Gian Marco Ghiggeri, Marco Pennesi, Alberto Edefonti, Rosanna Coppo, Francesco Emma

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)1487-1496
Number of pages10
JournalNephrology Dialysis Transplantation
Volume28
Issue number6
DOIs
Publication statusPublished - Jun 2013

Keywords

  • ACR criteria
  • Children
  • Chronic renal failure
  • Full house nephropathy
  • Lupus nephritis
  • Systemic lupus erythematosus

ASJC Scopus subject areas

  • Nephrology
  • Transplantation

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