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Therapeutic approaches for the treatment of renal disease in juvenile systemic lupus erythematosus: an international multicentre PRINTO study
  1. Paivi M Miettunen1,
  2. Angela Pistorio2,
  3. Elena Palmisani3,
  4. Angelo Ravelli3,4,
  5. Earl Silverman5,
  6. Sheila Oliveira6,
  7. Maria Alessio7,
  8. Ruben Cuttica8,
  9. Dimitrina Mihaylova9,
  10. Graciela Espada10,
  11. Srdjan Pasic11,
  12. Antonella Insalaco12,
  13. Seza Ozen13,
  14. Oscar Porras14,
  15. Flavio Sztajnbok15,
  16. Dragana Lazarevic3,
  17. Alberto Martini3,4,
  18. Nicolino Ruperto3,
  19. for the Paediatric Rheumatology International Trials Organisation (PRINTO)
  1. 1Division of Pediatric Rheumatology, University of Calgary, Alberta Children's Hospital, Calgary, Canada
  2. 2Servizio di Epidemiologia e Biostatistica, Istituto Giannina Gaslini, Genova, Italy
  3. 3Pediatria II Reumatologia, Istituto Giannina Gaslini, Genova, Italy
  4. 4Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy
  5. 5Division of Rheumatology, University of Toronto, Hospital for Sick Children, Toronto, Canada
  6. 6Universidade Federal do Rio de Janeiro, Instituto de Puericultura e Pediatria Martagao Gesteira, Rio de Janeiro, Brazil
  7. 7Dipartimento di Pediatria, Università di Napoli Federico II, Napoli, Italy
  8. 8Rheumatology Section, Hospital General de Niños Pedro de Elizalde, Buenos Aires, Argentina
  9. 9Department of Paediatric Rheumatology, University Children Hospital, Sofia, Bulgaria
  10. 10Sección Reumatologia, Hospital de Niños Dr Ricardo Gutierrez, Buenos Aires, Argentina
  11. 11Mother and Child Health Institute, Medical School, University of Belgrade, Belgrade, Serbia
  12. 12Reparto Pediatria II – Reumatologia, Ospedale Pediatrico Bambin Gesù, Roma, Italy
  13. 13Department of Pediatric Nephrology and Rheumatology, Hacettepe University Children's Hospital, Ankara, Turkey
  14. 14Servicio de Immunologia y Reumatologia Pediatrica, Hospital Nacional De Ninos Dr. Carlos Saenz Herrera, San Jose, Costa Rica
  15. 15Nucleo de Estudos da Saúde do Adolescente, Hospital Universitario Pedro Erneasto, Rio de Janeiro, Brazil
  1. Correspondence to Dr Nicolino Ruperto, Pediatria II—Reumatologia, Istituto Giannina Gaslini, Università di Genova, Pædiatric Rheumatology International Trials Organisation (PRINTO), EULAR Centre of Excellence in Rheumatology 2008-2013, Via Gaslini, 5, Genova 16147, Italy; nicolaruperto{at}, or


Objectives 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.

  • Lupus Nephritis
  • Disease Activity
  • Systemic Lupus Erythematosus
  • Treatment

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