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TABLE 101.5 CLASSIFICATION CRITERIA FOR JUVENILE SYSTEMIC SCLEROSIS (JSSc) Major criteria: Proximal skin sclerosis/induration of the skin (required) Minor criteria: At least criteria are required Skin: sclerodactyly Vascular: Raynaud phenomenon, abnormal nail fold capillaries, digital tip ulcers Gastrointestinal: dysphagia, gastroesophageal reflux Cardiac: arrhythmias, heart failure Renal: renal crisis, new onset hypertension Pulmonary: pulmonary fibrosis, low diffusing capacity, pulmonary hypertension Neurologic: neuropathy, carpal tunnel syndrome Musculoskeletal: tendon friction rubs, arthritis, myositis Serologic: Antinuclear antibody or SSc-selective antibodies (anticentromere, antitopoisomerase I [Scl-70], and others) From Zulian F, Woo P, Areytha BH, et al The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis Arthritis Rheum 2007;57(2):203–212 Copyright © 2007 by the American College of Rheumatology Adapted by permission of John Wiley & Sons, Inc Management Specific therapy for LSc and JSSc is divided into three categories: nonpharmacologic measures, disease-modifying therapy, and therapy for specific complications Nonpharmacologic measures include promoting physical activity, use of physiotherapy, use of corrective splints, and topical application of lanolin or water-soluble creams to the skin daily Pharmacologic therapy includes methotrexate, with or without the addition of oral or IV corticosteroids Cyclophosphamide appears to forestall pulmonary fibrosis if added early to the treatment regime Mycophenolate mofetil can be helpful for resistant disease If the esophageal sphincter is involved, patients should be advised to sleep with the head comfortably elevated, and an antacid may be prescribed Minor episodes of Raynaud syndrome are managed with prophylactic measures such as the avoidance of cold exposure and the use of warm clothing Biofeedback training and calcium-channel blockers may be helpful in decreasing the frequency of attacks Management of Complications and Emergencies Cardiac Complications Signs and symptoms of myocardial fibrosis are those of a cardiomyopathy with dyspnea, orthopnea, and fatigue ( Table 101.6 ) Angina pectoris and myocardial infarction may occur (see also Chapters 55 Pain: Chest and 86 Cardiac Emergencies ) Fibrosis of the conduction system may result in dysrhythmias,