Scleroderma: Not an orphan disease any more

Table 2 American College of Rheumatology/European League Against Rheumatism criteria for diagnosis of systemic sclerosis (2013) (Reproduced with permission)

Item	Sub-item(s)	Weight/score
Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (sufficient criterion)	-	9
Skin thickening of the fingers (only count the higher score)	Puffy Fingers	2
	Sclerodactyly of the fingers (distal to the 4 metacarpophalangeal joints but proximal to the proximal interphalangeal joints)	4
Fingertip lesions (only count the higher score)	Digital tip ulcers	2
	Fingertip pitting scars	3
Telangiectasia Abnormal nail fold capillaries		2
		2
Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)	Pulmonary arterial hypertension Interstitial lung disease	2
Raynaud’s phenomenon		3
SSc-related autoantibodies [anticentromere, anti-topoisomerase I (anti-Scl-70), anti-RNA polymerase III] (maximum score is 3)	Anticentromere	3
	Anti-topoisomerase I
	Anti-RNA polymerase III