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adverse outcomes, no randomized controlled treatment trials have been carried out in pediatric LS patients. Case series report that methotrexate (MTX) appears effective for treating pediatric patients, but the published protocols vary greatly, particularly regarding dose, route, and frequency of concomitant corticosteroid therapy4-7. Improvement has also been reported with calcipotriol and topical medications such as imiquimod and tacrolimus8-10, but whether pediatric rheumatologists use such therapies is unknown. To work toward developing a pediatric LS treatment trial, we carried out a survey to learn how pediatric rheumatologists (PR) in North America treat patients with LS. The survey included multiple-choice questions, open-ended options, and clinical vignettes. The survey followed the Padua Preliminary Classification Criteria of LS subtypes11 with the exception that our interest was in the more serious forms of LS, so we excluded plaque morphea (circumThe optimal treatment for localized scleroderma (LS), a disease associated with significant morbidity for the growing child, is unknown. Because the disease often begins in early or mid-childhood and commonly affects deep tissue layers, children are at risk for major growth problems such as limb length discrepancy and hemiatrophy. About 20% of pediDownloaded on November 15, 2021 from www.jrheum.org scribed superficial morphea) and asked about bullous morphea, which was not included in the Padua criteria. MATERIALS AND METHODS This survey was an effort of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Scleroderma Committee. CARRA is a North America pediatric rheumatology collaborative group. A Web-based survey (www.SurveyMonkey.com) was sent to all 195 pediatric rheumatology CARRA members in 2007. These 195 PR included 140 board-certified or senior PR (135 board-certified PR and 5 senior PR), 19 board-eligible PR, and 36 trainees (physicians in pediatric rheumatology fellowship programs). The majority of PR in North America are members of CARRA. In 2007, there were 249 North American members of the American College of Rheumatology who listed their specialty as pediatric rheumatology, including 10 clinicians who practiced both adult and pediatric rheumatology, 3 clinicians who practiced both allergy-immunology and rheumatology, and 50 trainees. The aim of the survey was to learn how PR use systemic immunosuppressive medications to treat the more serious forms of LS; the survey therefore did not ask about circumscribed superficial morphea (plaque morphea). Definitions of the different LS subtypes were provided from the Padua Preliminary Classification Criteria11: (1) circumscribed morphea (subtypes: superficial, deep), one or more oval or round lesions, with superficial lesions limited to epidermis and dermis, and deep lesions involving the subcutaneous tissue or panniculus; deep lesions often feeling taut and bound down; (2) linear scleroderma (subtypes: trunk/limbs, head), 1 or more linear lesions that can involve the underlying dermis, subcutaneous tissue, muscle, and bone; lesions on the head include en coup de sabre and Parry-Romberg syndrome; (3) generalized morphea, 4 or more large plaques (> 3 cm) that coalesce on at least 2 of 7 anatomic areas; the 7 areas are head/neck, right upper extremity, left upper extremity, right lower extremity, left lower extremity, anterior trunk, and posterior trunk; (4) pansclerotic morphea, deep generalized lesions that involve skin, subcutaneous tissue, muscle, and can involve bone; on extremities, there is circumferential involvement that spares fingertips and toes; no internal organ involvement for head or trunk lesions;
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