The Pediatric Rheumatology European Society/American College of Rheumatology/European League against rheumatism provisional classification criteria for juvenile systemic sclerosis

Francesco Zulian, Patricia Woo, Balu H. Athreya, Ronald M. Laxer, Thomas A. Medsger, Thomas J A Lehman, Marco Matucci Cerinic, Giorgia Martini, Angelo Ravelli, Ricardo Russo, Ruben Cuttica, Sheila Knupp Feitosa De Oliveira, Christopher P. Denton, Franco Cozzi, Ivan Foeldvari, Nicolino Ruperto

Research output: Contribution to journalArticlepeer-review

Abstract

Objective. To develop criteria for the classification of systemic sclerosis (SSc) in children (juvenile SSc). Methods. The study consisted of 3 phases: 1) collection of data on the signs and symptoms of actual patients with juvenile SSc that are useful for defining involvement of a particular organ; 2) selection of the parameters essential for the classification of juvenile SSc and preparation of a set of provisional classification criteria (PCC) using 2 Delphi surveys; 3) consensus conference consisting of 2 steps: discussion and rating of clinical profiles of 160 patients with definite juvenile SSc, possible juvenile SSc, or other fibrosing diseases as "having or not having juvenile SSc," using nominal group technique, and defining those PCC with the best statistical performance and highest face validity by using the clinical profiles of patients with definite juvenile SSc as the gold standard. Results. In phase 1, 55 centers submitted clinical data on 153 patients with juvenile SSc. A total of 48 signs and symptoms were derived from these patient data and were used to define 9 organ system categories (cutaneous, vascular, gastrointestinal, respiratory, renal, cardiac, neurologic, musculoskeletal, and serologic). During phase 2, these were reduced to 21 criteria (3 major criteria [Raynaud's phenomenon, proximal skin sclerosis/induration of the skin, and sclerodactyly] and 18 minor criteria) and combined to generate 86 different PCC. At the consensus conference, these 86 definitions were tested on the case profiles of 127 patients with juvenile SSc. The PCC with the highest ranking were proximal sclerosis/induration and at least 2 minor criteria. Conclusion. These provisional classification criteria for juvenile SSc will help standardize the conduct of clinical research, epidemiologic and outcome studies, and therapeutic trials.

Original languageEnglish
Pages (from-to)203-212
Number of pages10
JournalArthritis Care and Research
Volume57
Issue number2
DOIs
Publication statusPublished - Mar 15 2007

Keywords

  • Children
  • Classification criteria
  • Organ involvement
  • Scleroderma
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology

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