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Table 2 Revised jLS Study Entry Criteria: Inclusion, Exclusion, and Active Disease criteria

From: Developing comparative effectiveness studies for a rare, understudied pediatric disease: lessons learned from the CARRA juvenile localized scleroderma consensus treatment plan pilot study

Inclusion Criteria:

1. Localized scleroderma diagnosed by a Pediatric Rheumatologist or Pediatric Dermatologist according to Padua Preliminary Classification criteria; these criteria exclude eosinophilic fasciitis [23]

2. Fulfill active disease criteria:

 a. Either at least one item from Active disease criteria Group 1 or two from Group 2

3. Moderate to severe disease severity that warrants systemic therapy in the opinion of the treating physician

 a. Includes all subtypes that involve deeper tissue(s), extensive skin involvement, and/or extracutaneous involvement

4. Age < 18 years at onset of disease

5. Age < 21 years at onset of treatment

Exclusion criteria:

1. Treated with systemic corticosteroids in the prior 2 weeks

2. Treated with methotrexate or mycophenolate mofetil within the prior 4 weeks

3. Another defined systemic rheumatic disease (e.g., systemic sclerosis)

4. Intolerance to study medications

Revised Active Disease criteria

Group 1:

Developing comparative effectiveness studies

1. New, larger, or deeper lesion that developed within the past 3 months associated with erythema, violaceous color, and/or skin thickening. Disease extension must be documented by one of the following

 a. Clinical exam

 b. Measurements or tracings

 c. Photographs (may be provided by family)

 d. Imaging: MRI, ultrasound, CT, 3D imager

2. Erythema of moderate or severe level

 a. Erythema scoring level based upon LS scoring atlas

3. Violaceous color

 a. Can range from lilac ring to deep violaceous color

Group 2:

1. Erythema of mild level

 a. Erythema scoring level based upon LS scoring atlas

2. Waxy white or yellow lesion

 a. These lesions have white or yellowish appearance with smooth, waxy feeling

 b. They are associated with skin thickening (induration)

3. Skin thickening of lesion

 a. Assessed according to modified Rodnan Skin Scoring (mRSS) levels [15]

4. Tactile warmth of the lesion

 a. Examiner appreciation of temperature difference based upon comparison to control site (unaffected contralateral site if available).

5. Worsening hair loss on scalp or face

6. Inflammation within lesion identified on tissue biopsy

  1. Revised entry criteria for jLS treatment studies. Modifications of the criteria used for the jLS Consensus Treatment Plan (CTP) Pilot study shown in Table 1; modified criteria are shown in italics. These criteria are not intended to qualify or disqualify patients for any specific treatment. The LS Scoring Atlas was generated by the LS workgroup of CARRA and contains photographs of patient lesions demonstrating the different visible scored features; ≥ 80% consensus agreement by workgroup members was required for the photograph to be included in the atlas [20]
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Pediatric Rheumatology

ISSN: 1546-0096