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


CONCLUSIONS
These provisional classification criteria for juvenile SSc will help standardize the conduct of clinical research, epidemiologic and outcome studies, and therapeutic trials.
Zulian
Francesco
F
Dipartimento di Pediatria, University of Padua, Padua, Italy. zulian@pediatria.unipd.it
Woo
Patricia
P
Athreya
Balu H
BH
Laxer
Ronald M
RM
Medsger
Thomas A
TA
Lehman
Thomas J A
TJ
Cerinic
Marco Matucci
MM
Martini
Giorgia
G
Ravelli
Angelo
A
Russo
Ricardo
R
Cuttica
Ruben
R
de Oliveira
Sheila Knupp Feitosa
SK
Denton
Christopher P
CP
Cozzi
Franco
F
Foeldvari
Ivan
I
Ruperto
Nicolino
N
D002648:Child; D003697:Delphi Technique; D006801:Humans; D010372:Pediatrics / Q000592:standards*; D017410:Practice Guidelines as Topic; D012219:Rheumatology / Q000592:standards*; D012595:Scleroderma, Systemic / Q000145:classification* / Q000175:diagnosis*; D012955:Societies, Medical