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more aggressively (age < 3 years 22%, 3–6 years 20%, 7–10 years 15%, 11–14 years 14%, > 15 years 1%). Similarly, the majority did not vary treatment in response to patient developmental stage (70%, 87/124); among the 30% who did, prepubertal (27%) or pubertal (20%) patients were treated more aggressively than those who were postpubertal. Features of disease activity and remission. For these questions, we did not define disease activity or remission because definitions for these terms do not currently exist for LS. Instead, we sought to elicit the characteristics that PR thought represent active and inactive disease, and how long the disease should appear inactive before the PR thinks the disease is in remission. PR were asked to rank features indicative of disease activity from 0 (irrelevant) to 5 (important indicator). Six features were rated as high activity features: new lesion, extension of an existing lesion, erythema, swelling or induration of lesion margin, violaceous color, and lesion edge warmth (mean scores 4.78, 4.62, 4.55, 4.40, 4.24, and 4.05, respectively). These features were significantly higher than distinct margin, subcutaneous atrophy, hyperpigmentation, dermal appendage atrophy, skin atrophy, or hypopigmentation (mean scores < 2.5). Additional high activity features listed by some PR were elevated acute-phase reactants, eosinophilia, discomfort, hidebound feel, and calcinosis. PR were asked which features needed to be present before they would stop LS treatment. Most required the absence of features that they felt indicated activity, namely absence of new lesions (99%, 123/124), stable lesion size or shrinkage (98%, 121/124), lack of erythema (94%, 116/124), lack of violaceous coloration (87%, 108/124), and flat margins or resolution of marginal induration (77%, 96/124). The majority (60%, 74/124) also required resolution of laboratory indices of inflammation, while 22% (27/124) of respondents required completion of linear growth. When asked the duration of time elapsed since the last indication of active disease before they would consider a patient in remission on medications, most specified 12 months (40%, 50/124) or 6 months (31%, 38/124). Complete remission required 12 months (54%, 67/124) or 24 months (28%, 35/124) of inactive disease off all medications. However, 8% said they did not know when complete remission was achieved. Clinical vignettes. To learn if most PR follow similar treatment protocols, PR were asked to detail their treatment of clinical vignettes. PR were asked to assume that the presented vignettes were similar to average patients who fit the vignette history, and to assume that the vignette patients had an average response to treatment. All respondents were given the same vignette of a 4-year-old girl with a 2-month history of a linear LS lesion on her right lateral leg extending across her ankle. Her lesion was initially swollen, with an erythematous-violaceous border, and had warmth and subcutaneous tissue loss at the time of her visit. Her laboratory studies show an elevated erythrocyte sedimentation rate. All respondents would treat this vignette patient with MTX, with most using the subcutaneous route (57%, 65/114; Table 1). The other respondents were equally divided between using oral MTX and not having a preference for route of administration. The majority of respondents calculated MTX dose based on body weight (mg/kg). For the 27% of respondents who calculated dose based on body surLi, et al: Pediatric localized scleroderma 177 Personal non-commercial use only. The Journal of Rheumatology Copyright © 2010. All rights reserved. Downloaded on November 15, 2021 from www.jrheum.org face area (mg/m2), the most frequently
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