Provisional classification criteria for juvenile systemic sclerosis by the Pediatric Rheumatology European Society, the American College of Rheumatology, and the European League against Rheumatism

Major criterion	Proximal sclerosis/induration of the skin
Minor criteria	Skin	Sclerodactyly
	Vascular	Raynaud phenomenon
		Nailfold capillary abnormalities
		Digital tip ulcers
	Gastrointestinal	Dysphagia
		Gastroesophageal reflux
	Renal	Renal crisis
		New-onset arterial hypertension
	Cardiac	Arrhythmias
		Heart failure
	Respiratory	Pulmonary fibrosis (seen on HRCT or radiograph of the chest)
		DLCO
		Pulmonary hypertension
	Musculoskeletal	Tendon friction rubs
		Arthritis
		Myositis
	Neurologic	Neuropathy
		Carpal tunnel syndrome
	Serology	Antinuclear antibodies
		SSc selective autoantibodies (anticentromere, antitopoisomerase I, antifibrillarin, anti-PM/Scl, antifibrillin, or anti-RNA polymerase I or III)

In a patient less than 16 years of age, juvenile systemic sclerosis is diagnosed if the major criterion and at least 2 of 20 minor criteria are present.

HRCT: high-resolution computed tomography; DLCO: diffusing capacity of the lung for carbon monoxide; SSc: systemic sclerosis; anti-PM/Scl: anti-polymyositis/scleroderma antinuclear antibodies, also called anti-exosome antibodies.

Reproduced with permission from: Zulian F, Woo P, Athreya 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; 57:203. Copyright © 2007 Wiley-Liss, Inc. a subsidiary of John Wiley & Sons Inc.

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Provisional classification criteria for juvenile systemic sclerosis by the Pediatric Rheumatology European Society, the American College of Rheumatology, and the European League against Rheumatism