Abstract
BackgroundOur earlier work in the ultrasonograpy of localized scleroderma (LS) suggests that altered levels of echogenicity and vascularity can be associated with disease activity. Utrasound is clinically benign and readily available, but can be limited by operator dependence. We present our efforts to standardize image acquisition and interpretation of pediatric LS to better evaluate the correlation between specific sonographic findings and disease activity.MethodsSeveral meetings have been held among our multi-center group (LOCUS) to work towards standardizing sonographic technique and image interpretation. Demonstration and experience in image acquisition were conducted at workshop meetings. Following meetings in 2007, an ultrasound measure was developed to standardize evaluation of differences in echogenicity and vascularity. Based upon our initial observations, we have labeled this an ultrasound disease activity measure. This preliminary measure was subsequently evaluated on over 180 scans of pediatric LS lesions. This review suggested that scoring levels should be expanded to better capture the range of observed differences. The revised levels and their definitions were formulated at a February 2009 workshop meeting. We have also developed assessments for scoring changes in tissue thickness and lesion size to better determine if these parameters aid evaluation of disease state.ResultsWe have standardized our protocol for acquiring ultrasound images of pediatric LS lesions. A wide range of sonographic differences has been seen in the dermis, hypodermis, and deep tissue layers of active lesions. Preliminary ultrasound assessments have been generated. The disease activity measure scores for altered levels of echogenicity and vascularity in the lesion, and other assessments score for differences in lesion tissue layer thickness and changes in lesion size.ConclusionsWe describe the range of sonographic differences found in pediatric LS, and present our efforts to standardize ultrasound acquisition and image interpretation for this disease. We present ultrasound measures that may aid evaluation of disease state. These assessments should be considered a work in progress, whose purpose is to facilitate further study in this area. More studies are needed to assess their validity and reliability.
Highlights
Our earlier work in the ultrasonograpy of localized scleroderma (LS) suggests that altered levels of echogenicity and vascularity can be associated with disease activity
Several groups have reported that changes in dermal thickness and level of echogenicity correlate with disease activity [6,7,8]
Development of the Disease activity measure Our earlier work suggested that altered echogenicity and vascularity signals in ultrasound scans of localized scleroderma patients can be associated with disease activity [9,10]
Summary
Standardization of image acquisition carried out Two meetings were held at HUMC in 2007 (5/11/07, 7/ 22/07) to demonstrate image acquisition, and allow LOCUS radiologists and sonographers to review LS images, and practice image acquisition on patient volunteers. A preliminary technique protocol was developed by MSL, AM, and SCL This protocol was reviewed and modified at the Feb 21-24, 2009 meeting, with demonstrations and supervised image acquisition sessions on patient volunteers over three days. Standardization of image interpretation During the 2007 LOCUS meetings, radiologists, sonographers, and clinicians reviewed ultrasound images acquired from 8 LS patients followed at Hackensack University Medical Center (HUMC), comparing active to inactive patients. The meeting began with a review of the previously generated ultrasound disease activity measure, and included examples from HUMC LS patient scans of the scoring levels of each parameter of the measure. Additional patient images were reviewed that varied between each other in observable degree of echogenicity or color Doppler differences, but would still be scored at the same level according to the developed measure. This study was approved by the HUMC institutional review board and is endorsed by CARRA
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