Criteria

Score

Comments

Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints

9

Sufficient criterion

Skin thickening of the fingers

Sclerodactyly of the fingersb

4

Fingertip lesionsc

Fingertip pitting scars

3

Telangiectasiad

2

Abnormal nail-fold capillariese

2

Pulmonary arterial hypertensionf and/or interstitial lung diseaseg

2

Raynaud phenomenonh

3

SSc-related autoantibodies:

Interpretation: The diagnosis of SSc can be made in patients with a total score ≥9.

a A diffuse, usually nonpitting increase in soft tissue mass of the digits extending beyond the normal confines of the joint capsule, which affects the physiological contour of the fingers (normal digits are tapered distally with the tissues following the contours of the digital bone and joint structures).

b Skin hardening distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints.

c Ulcers 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 ischemia, rather than trauma or exogenous causes.

d Telangiectasias are visible, macular, dilated superficial blood vessels, which collapse upon pressure and fill slowly when pressure is released. Telangiectasias in a scleroderma-like pattern are round, well-demarcated, and found on hands, lips, inside of the mouth, and/or are large mat-like telangiectasias. Telangiectasias should be distinguished from rapidly filling spider angiomas with a central arteriole and from dilated superficial vessels.

e Abnormal nail-fold capillary patterns consistent with systemic sclerosis are enlarged capillaries and/or capillary loss with or without pericapillary hemorrhages at the nail fold. They may also be seen on the cuticle.

f Pulmonary arterial hypertension diagnosed by right-sided heart catheterization according to standard definitions.

g Interstitial lung disease: Pulmonary 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.

h Raynaud phenomenon: Self-reported or reported by a physician, with at least a 2-phase color 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.

The criteria are not applicable to patients with skin thickening sparing the fingers or to patients who have a scleroderma-like disorder that better explains their manifestations (eg, nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromelalgia, porphyria, lichen sclerosis, graft-versus-host disease, diabetic cheiroarthropathy).