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The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis
  1. Dragana Lazarevic1,
  2. Angela Pistorio2,
  3. Elena Palmisani1,
  4. Paivi Miettunen1,
  5. Angelo Ravelli1,3,
  6. Clarissa Pilkington4,
  7. Nico M Wulffraat5,
  8. Clara Malattia3,
  9. Stella Maris Garay6,
  10. Michael Hofer7,
  11. Pierre Quartier8,
  12. Pavla Dolezalova9,
  13. Inmaculada Calvo Penades10,
  14. Virginia P L Ferriani11,
  15. Gerd Ganser12,
  16. Ozgur Kasapcopur13,
  17. Jose Antonio Melo-Gomes14,
  18. Ann M Reed15,
  19. Malgorzata Wierzbowska16,
  20. Lisa G Rider17,
  21. Alberto Martini1,2,3,
  22. Nicolino Ruperto1,
  23. for the Paediatric Rheumatology International Trials Organisation (PRINTO)
  1. 1Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genova, Italy
  2. 2Servizio di Epidemiologia e Biostatistica, Istituto Giannina Gaslini, Genova, Italy
  3. 3Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy
  4. 4Centre of Paediatric and Adolescent Rheumatology, Great Ormond Street Hospital, London, UK
  5. 5Department of Pediatric Immunology and Rheumatology, Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands
  6. 6Unidad de Reumatologia, Hospital Sor Maria Ludovica, La Plata, Argentina
  7. 7Pediatric Rheumatology of Western Switzerland Lausanne University Hospital, Lausanne, and Geneva University Hospital, Lausanne, Switzerland
  8. 8Centre de référence national pour les Arthrites Juveniles, Unité d’Immunologie, Hématologie et Rhumatologie Pediatrique, Université Paris-Descartes and Hôpital Necker-Enfants Malades, Paris, France
  9. 9Department of Pediatrics and Adolescent Medicine, Charles University in Prague and General University Hospital, Praha 2, Czech Republic
  10. 10Unidad de Reumatologia Pediatrica, Hospital Universitario La Fe, Valencia, Spain
  11. 11Department of Pediatrics, School of Medicine of Ribeirao Preto, University of Sao Paulo, Ribeirao Preto, Brazil
  12. 12Center of Rheumatology North-West Germany, Clinic for Pediatric Rheumatology, St.Josef Stift Sendenhorst, Westtor 7, Germany
  13. 13Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey
  14. 14Childhood Adolescent and Young Adult Outpatients Clinic of Rheumatology, Instituto Portugues de Reumatologia, Lisbon, Portugal
  15. 15Rheumatology, Mayo Clinic School of Medicine and Mayo Foundation, Rochester, Minnesota, USA
  16. 16Paediatric Clinic, Institute of Rheumatology, Warsaw, Poland
  17. 17NIEHS, NIH, HHS, Environmental Autoimmunity Group, Bethesda, Maryland, USA
  1. Correspondence to Nicolino Ruperto, Istituto Giannina Gaslini, Pediatria II, Reumatologia, PRINTO, Largo Gaslini 5, Genova 16147, Italy; nicolaruperto{at}ospedale-gaslini.ge.it

Abstract

Objectives To develop data-driven criteria for clinically inactive disease on and off therapy for juvenile dermatomyositis (JDM).

Methods The Paediatric Rheumatology International Trials Organisation (PRINTO) database contains 275 patients with active JDM evaluated prospectively up to 24 months. Thirty-eight patients off therapy at 24 months were defined as clinically inactive and included in the reference group. These were compared with a random sample of 76 patients who had active disease at study baseline. Individual measures of muscle strength/endurance, muscle enzymes, physician's and parent's global disease activity/damage evaluations, inactive disease criteria derived from the literature and other ad hoc criteria were evaluated for sensitivity, specificity and Cohen's κ agreement.

Results The individual measures that best characterised inactive disease (sensitivity and specificity >0.8 and Cohen's κ >0.8) were manual muscle testing (MMT) ≥78, physician global assessment of muscle activity=0, physician global assessment of overall disease activity (PhyGloVAS) ≤0.2, Childhood Myositis Assessment Scale (CMAS) ≥48, Disease Activity Score ≤3 and Myositis Disease Activity Assessment Visual Analogue Scale ≤0.2. The best combination of variables to classify a patient as being in a state of inactive disease on or off therapy is at least three of four of the following criteria: creatine kinase ≤150, CMAS ≥48, MMT ≥78 and PhyGloVAS ≤0.2. After 24 months, 30/31 patients (96.8%) were inactive off therapy and 69/145 (47.6%) were inactive on therapy.

Conclusion PRINTO established data-driven criteria with clearly evidence-based cut-off values to identify JDM patients with clinically inactive disease. These criteria can be used in clinical trials, in research and in clinical practice.

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Footnotes

  • Funding Supported by grants from the European Union (contract no. QLG1-CT-2000-00514) and IRCCS G. Gaslini, Genoa, Italy. DL was the recipient of the European League Against Rheumatism (EULAR) Scientific Training Bursaries. PMM attended the PRINTO headquarter at Gaslini Hospital in Genoa (Italy) as part of her visiting professorship sabbatical. LGR was supported by the intramural research program of the National Institute of Environmental Health Sciences, National Institutes of Health.

  • Competing interests None.

  • Ethics approval Ethics approval was obtained from the ethics committees of the participating centres.

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

  • Data sharing statement The PRINTO database related to this project is open to further research prior to acceptance of the proposal by the PRINTO Advisory Council (www.printo.it).

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