title: Therapeutic approaches for the treatment of renal disease in juvenile systemic lupus erythematosus: an international multicentre PRINTO study.
abstract: OBJECTIVE
  To evaluate therapeutic approaches and response to therapy in juvenile systemic lupus erythematosus (SLE) with renal involvement in a large prospective international cohort from four geographic areas.
  
  
  METHODS
  New onset and flared patients with active renal disease (proteinuria ≥0.5 g/24 h) were enrolled in 2001-2004. Therapeutic approaches and disease activity parameters were analysed at baseline, 6, 12 and 24 months. Response was assessed by the PRINTO/ACR criteria.
  
  
  RESULTS
  218/557 (79.8% female subjects, 117 new onset and 101 flared) patients with active renal disease were identified; 66 patients were lost to follow-up and 11 died. Mean age at disease onset for new onset group was higher than for flared group (13.1 vs 10.2 years, p<0.0001). At baseline, both groups had similar renal activity with similar median doses of corticosteroids (1.0-0.76 mg/kg/day). Cyclophosphamide (43.1%) and azathioprine (22%) were the most common immunosuppressive drugs. At baseline, South American patients received higher doses of corticosteroids than in other areas in new onset (median 1.16 vs 0.8-1 mg/kg/day) while cyclophosphamide use was similar in all four regions in the new onset group. There were no differences regarding the use of azathioprine or mycophenolate mofetil worldwide. PRINTO 70 response was reached in a greater percentage of new onset versus flared patients (74.8% vs 53.3%; p=0.005) at 6 months while at 24 months ACR 90 was reached by 69.9% and 56.1%, respectively.
  
  
  CONCLUSIONS
  New onset and flared juvenile SLE improved similarly over 24 months with minimal differences in therapeutic approaches worldwide.
authors.affiliation: Division of Pediatric Rheumatology, University of Calgary, Alberta Children's Hospital, Calgary, Canada.
authors.forename: Paivi M
authors.initials: PM
authors.lastname: Miettunen
authors.forename: Angela
authors.initials: A
authors.lastname: Pistorio
authors.forename: Elena
authors.initials: E
authors.lastname: Palmisani
authors.forename: Angelo
authors.initials: A
authors.lastname: Ravelli
authors.forename: Earl
authors.initials: E
authors.lastname: Silverman
authors.forename: Sheila
authors.initials: S
authors.lastname: Oliveira
authors.forename: Maria
authors.initials: M
authors.lastname: Alessio
authors.forename: Ruben
authors.initials: R
authors.lastname: Cuttica
authors.forename: Dimitrina
authors.initials: D
authors.lastname: Mihaylova
authors.forename: Graciela
authors.initials: G
authors.lastname: Espada
authors.forename: Srdjan
authors.initials: S
authors.lastname: Pasic
authors.forename: Antonella
authors.initials: A
authors.lastname: Insalaco
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.forename: Oscar
authors.initials: O
authors.lastname: Porras
authors.forename: Flavio
authors.initials: F
authors.lastname: Sztajnbok
authors.forename: Dragana
authors.initials: D
authors.lastname: Lazarevic
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
authors.forename: Nicolino
authors.initials: N
authors.lastname: Ruperto
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001379:Azathioprine / Q000627:therapeutic use*; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006304:Health Status; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007391:International Cooperation; D008181:Lupus Nephritis / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology / Q000652:urine; D008297:Male; D011446:Prospective Studies; D011507:Proteinuria / Q000473:pathology; D012008:Recurrence; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine
keywords: Disease Activity; Lupus Nephritis; Systemic Lupus Erythematosus; Treatment