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| Name | Class |
|---|---|
| Childhood Arthritis and Rheumatology Research Alliance | OTHER |
| Joseph Sanzari Children's Hospital | UNKNOWN |
| University of Pittsburgh | OTHER |
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Localized scleroderma (LS) is a skin disease that can cause complications such as disfigurement and limitation of mobility. Treatment for LS should be initiated early in the disease, as late-stage scleroderma does not respond well to treatment. Objective tools for diagnosis and follow-up of treatment for LS are not available clinically and are urgently needed. A new non-invasive ultrasound technique called "elastography" measures stiffness of tissues and holds potential for providing objective measures for follow-up of treatment of LS in children and adolescents. The team plans to conduct a multicenter study in North America to determine whether elastography ultrasound can show changes in skin stiffness for a period of 9 to 12 months after initiation of treatment for LS. This novel technology may add information to the existing imperfect clinical tools, thus improving the way therapy is offered to patients with LS.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Elastography group | Boys and girls 0-18 years of age at the time of disease onset with a new or existing diagnosis of localized scleroderma will have a physical examination and elastogrpahy ultrasound at specific timepoints, based on the timeframe for clinical visits. A questionnaire will be administered by the time of each study visit. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Elastography Ultrasound | Diagnostic Test | The US imaging protocol will include three parts: (i) gray-scale; (ii) color Doppler, and (iii) Shear-wave elastography (SWE) |
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| Measure | Description | Time Frame |
|---|---|---|
| Skin stiffness | Skin stiffness will be assessed using Elastography (SWE) Ultrasound, a sonographic technique that uses mechanical waves to assess tissue elasticity and display it in a quantitative manner. The stiffness of the tissue is directly proportional to its shear wave velocity. SWE will measure interval changes of skin stiffness during treatment at the target lesion and the unaffected contralateral site. | Baseline, 3-6m and 9-12m |
| Measure | Description | Time Frame |
|---|---|---|
| Lesion activity responsiveness | The LoSCAT (Localized Sclerodema Cutaneous Assessment Tool scores) assesses 18 cutaneous anatomic sites, capturing both disease activity (mLoSSI - Modified Localized Skin Severity Index) and disease damage (LoSDI - Localized Scleroderma Damage Index). Scores for each site are based on the most severe score for each parameter ranging from 0 to 3. A higher score represents a worse outcome. |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with diagnosis of LS
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| The Hospital for Sick Children | Toronto | Ontario | M5G 1X8 | Canada |
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| Label | URL |
|---|---|
| CARRA is a registered non-profit organization. Its mission is to conduct collaborative research to prevent, treat and cure pediatric rheumatic diseases. | View source |
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| ID | Term |
|---|---|
| D045743 | Scleroderma, Diffuse |
| ID | Term |
|---|---|
| D012595 | Scleroderma, Systemic |
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
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| The Localized Scleroderma Quality of Life Instrument (LoSQI ) | Other | The questionnaire is a validated disease-specific patient-reported outcome measure that covers different domains (skin sensations, physical functioning, body image and social support, designed for children and adolescents with localized scleroderma |
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| Baseline, 3-6m and 9-12m |
| Echogenicity scores for dermis and hypodermis | Standard ultrasound protocol. Identifying sonographic changes in the involved body site will be accomplished by comparing both echogenicity and color Doppler signal to a corresponding normal site. The score is determined by summing the absolute value of the individual parameters, with a range from 0 to 15 (maximum echogenicity score of 6, maximum vascularity score of 9). A higher score represents a worse outcome. | Baseline, 3-6m and 9-12m |
| Vascularity scores for dermis and hypodermis | Doppler Ultrasound protocol. Identifying sonographic changes in the involved body site will be accomplished by comparing both echogenicity and color Doppler signal to a corresponding normal site. The score is determined by summing the absolute value of the individual parameters, with a range from 0 to 15 (maximum echogenicity score of 6, maximum vascularity score of 9). A higher score represents a worse outcome. | Baseline, 3-6m and 9-12m |
| Quality of Life | Completiom of the LoSQI (Localized Scleroderma Quality of Life Instrument), a self-reported disease-specific questionnaire to assess the quality of life of patients (children, adolescents and young adults) with localized scleroderma. It consists of 21 items across three theoretical domains: (1) skin sensations, (2) physical functioning and musculoskeletal sequelae, and (3) body image and social support. Scores are based on the most severe score for each parameter ranging from 0 to 3. The questionnaire includes questions that comprise the Pain & Physical Functioning subscale, and the Body Image & Social Support subscale. Scores are summoned and converted to a scale, ranging from 41.3 to 71.6 and 43.58 to 68.42, respectively. A higher score represents a worse outcome. | Baseline, 3-6m and 9-12m |