Scleroderma: Not an orphan disease any more

Table 1 American College of Rheumatology 1980 criteria for systemic sclerosis

Major criterion	Minor criterion
Scleroderma like skin change proximal to MCP or MTP joints	Sclerodactyly
	Digital pitting scars of fingertips or loss of distal finger pad
	Bibasilar Pulmonary fibrosis

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