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Extended report
A longitudinal PRINTO study on growth and puberty in juvenile systemic lupus erythematosus
  1. Marite Rygg1,
  2. Angela Pistorio2,
  3. Angelo Ravelli3,
  4. Mohamad Maghnie4,
  5. Natascia Di Iorgi4,
  6. Brigitte Bader-Meunier5,
  7. Carlos Da Silva6,
  8. Rosa Roldan-Molina7,
  9. Judith Barash8,
  10. Cristina Dracou9,
  11. Sylvie Gandon Laloum10,
  12. Katerina Jarosova11,
  13. Chantal Job Deslandre12,
  14. Isabelle Koné-Paut13,
  15. Franco Garofalo14,
  16. Joseph Press15,
  17. Claudia Sengler16,
  18. Tsivia Tauber17,
  19. Alberto Martini3,
  20. Nicolino Ruperto18 for the Paediatric Rheumatology International Trials Organisation (PRINTO)
  1. 1Faculty of medicine, Department of Laboratory Medicine, Children's and Women's Health, Norwegian University of Science and Technology and Department of Pediatrics, St. Olavs Hospital, Trondheim, Norway
  2. 2IRCCS G Gaslini, Servizio di Epidemiologia e Biostatistica, Genova, Italy
  3. 3IRCCS G Gaslini, Pediatria II and Università degli Studi di Genova, Genova, Italy
  4. 4IRCCS G. Gaslini, Department of Paediatrics, University of Genova, Genova, Italy
  5. 5Centre de Référence, National “Arthrites Juvéniles”, Hôpital Necker-Enfants Malades, APHP, Paris, France
  6. 6Department of Pediatrics, Hospital de Clinicas de Faculdades de Medicina da Universidade Federal de Uberlandia, Uberlândia, Brazil
  7. 7Unidad de Reumatologia, Reina Sofia Hospital, Cordoba, Spain
  8. 8Pediatric Day Care, Kaplan Medical Center, Rehovot, Israel
  9. 92nd Department of Paediatrics, NHS, P. A. Kyriakou Childrens Hospital, Athens, Greece
  10. 10Chirurgie Pediatrique, Chu Caen FEH, Caen, France
  11. 11Clinical department, Revmatologicky Ustav, Praha 2, Czech Republic
  12. 12Service Rhumatologie A, Hopital Cochin Centre de reference national pour les Arthrites Juveniles, Paris, France
  13. 13Centre de Référence National des Maladies auto-inflammatoires, rhumatologie pediatrique, CHU Le Kremlin Bicetre (University of Paris SUD), Paris, France
  14. 14Struttura Complessa di Pediatria, Ospedale degli Infermi di Biella, Biella, Italy
  15. 15Division of Pediatrics, Soroka Univ Medical Center, Beer-Sheva, Israel
  16. 16Kinderklinik Rheumatologie, Charite University Hospital Berlin, Berlin, Germany
  17. 17Pediatric Rheumatology Clinic, Assaf Harofe Medical Center, Zrifin, Israel
  18. 18IRCCS G Gaslini, Pediatria II-PRINTO Reumatologia, Genova, Italy
  1. Correspondence to Nicolino Ruperto, Pædiatric Rheumatology International Trials Organisation (PRINTO), IRCCS G. Gaslini, Università di Genova, Pediatria II – Reumatologia, EULAR Centre of Excellence in Rheumatology 2008-2013, Largo Gaslini, 5, 16147 Genova, Italy; nicolaruperto@


Objective To obtain longitudinal data on growth/puberty in a large-scale, multi-national prospective cohort of juvenile systemic lupus erythematosus (SLE).

Methods Data from 331/557 (59.4%) patients ≤18 years old with juvenile SLE in active phase, with anthropometric data available at four follow-up visits, were studied.

Results There was a significant reduction in parent-adjusted height z score with time in females and males (p<0.0001), with a significant gender difference (p<0.0001) and with male height being most affected. Median body mass index z score peaked at 6 months and was still significantly above baseline after 26 months (p<0.01), with no gender difference. Standardised height reduction was inversely related to age at onset. Females with onset age <12 years had a median parent-adjusted height z score of −0.87 with no catch-up growth. At the end of the study, growth failure was seen in 14.7% of the females and 24.5% of the males. Height deflection (less than −0.25/year) was found in 20.7% of the females and 45.5% of the males. Delayed pubertal onset was seen in 15.3% and 24% of the females and males, respectively, and delayed/absent menarche was seen in 21.9%, while 36.1% of the females and 44% of the males had some degree of delayed pubertal development. Growth failure baseline determinants were previous growth failure (OR: 56.6), age at first visit ≤13.4 years (OR: 4.2) and cumulative steroid dose >426 mg/kg (OR: 3.6).

Conclusions The children at risk of having a negative effect on height and pubertal development are prepubertal and peripubertal children treated with >400 mg/kg cumulative dose of corticosteroids.

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  • Funding Funded by a grant from the European Union (contract no. QLG1-CT-2000-00514) and by IRCCS G. Gaslini, Genoa, Italy.

  • Competing interest None.

  • Ethics approval Ethics committees of participating centres as appropriate.

  • Provenance and peer review Not commissioned; externally peer reviewed.