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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 2 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,