Table 2 Diagnostic criteria for systemic sclerosis (American College of Rheumatology/European League Against Rheumatism criteria for the classification of systemic sclerosis [9])

Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (sufficient criteria)

-

9

Skin thickening of the fingers (only count the higher score)

Puffy fingers

Sclerodactly of the fingers (distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints)

2

4

Fingertip lesions (only count the higher score)

Distal tip ulcers

Fingertip pitting scars

2

3

Telangiectasia

-

2

Abnormal nailfold capillaries

-

2

Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)

Pulmonary arterial hypertension

Interstitial lung disease

2

2

Raynaud’s phenomenon

-

3

Systemic sclerosis-related autoantibodies

Anticentromere

Anti-topoisomerase I (anti-Scl 70)

Anti-RNA polymerase III

3