Table 2

Definitions of items/sub-items in the American College of Rheumatology/European League Against Rheumatism criteria for the classification of systemic sclerosis

ItemDefinition
Skin thickeningSkin thickening or hardening not due to scarring after injury, trauma, etc.
Puffy fingersSwollen digits–a diffuse, usually nonpitting increase in soft tissue mass of the digits extending beyond the normal confines of the joint capsule. Normal digits are tapered distally with the tissues following the contours of the digital bone and joint structures. Swelling of the digits obliterates these contours. Not due to other causes such as inflammatory dactylitis.
Fingertip ulcers or pitting scarsUlcers or scars distal to or at the proximal interphalangeal joint not thought to be due to trauma. Digital pitting scars are depressed areas at digital tips as a result of ischaemia, rather than trauma or exogenous causes.
TelangiectasiaTelangiectasiae are visible macular dilated superficial blood vessels, which collapse upon pressure and fill slowly when pressure is released. Telangiectasiae in a scleroderma-like pattern are round and well demarcated and found on hands, lips, inside of the mouth, and/or are large mat-like telangiectasiae. Distinguishable from rapidly filling spider angiomas with central arteriole and from dilated superficial vessels.
Abnormal nailfold capillary pattern consistent with systemic sclerosisEnlarged capillaries and/or capillary loss with or without pericapillary haemorrhages at the nailfold. May also be seen on the cuticle.
Pulmonary arterial hypertensionPulmonary arterial hypertension diagnosed by right-sided heart catheterisation according to standard definitions.
Interstitial lung diseasePulmonary fibrosis seen on high-resolution CT or chest radiography, most pronounced in the basilar portions of the lungs, or occurrence of ‘Velcro’ crackles on auscultation, not due to another cause such as congestive heart failure.
Raynaud's phenomenonSelf-reported or reported by a physician, with at least a 2-phase colour change in finger(s) and often toe(s) consisting of pallor, cyanosis, and/or reactive hyperemia in response to cold exposure or emotion; usually one phase is pallor.
SSc-related auto antibodiesAnticentromere antibody or centromere pattern seen on antinuclear antibody testing, anti–topoisomerase I antibody (also known as anti–Scl-70 antibody), or anti– RNA polymerase III antibody. Positive according to local laboratory standards.
  • SSc, systemic sclerosis.