Table 3.

Proposed item definitions

Item	Definition
Abnormal nailfold capillary pattern consistent with SSc	Enlarged capillaries and/or capillary loss with or without peri-capillary hemorrhages.
Anti-centromere antibody or centromere pattern on antinuclear antibody test	Positive according to local laboratory standards
Anti-topoisomerase I antibody	Positive according to local laboratory standards
Antinuclear antibody	Positive according to local laboratory standards
Anti-PM-Scl antibody	Positive according to local laboratory standards
Anti-RNA polymerase III antibody	Positive according to local laboratory standards. May not be available in all labs as part of extractable nuclear antigen (ENA)
Calcinosis	Detected either clinically or by radiographically. Calcinosis is defined as palpable, dermal and/or subcutaneous or intramuscular deposits. It is usually located in digits or over large proximal joints or extensor surfaces of distal extremities.
Reduced carbon monoxide diffusion capacity (DLCO) percent predicted	According to local laboratory standards, <80% of predicted or lower cut point.
Digital pulp loss or acro-osteolysis	Loss of substance from the fingertip pad, as a result of ischemia rather than trauma or exogenous cause (27). Acroosteolysis – osteolysis of the distal phalanx/phalanges (28)
Dysphagia for solid foods	By history, as substernal discomfort on swallowing or sensation of food being held up or “stuck” in a retrosternal location.
Esophageal dilatation	Esophagus dilatated by imaging (barium swallow, chest radiograph, or high resolution computed tomography [HRCT] of the chest).
Finger flexion contractures	Inability to extend fingers to neutral position due to skin or tendon tightening, not due to arthritis deformities, Dupuytren’s or other conditions.
Finger tip ulcers or pitting scars	Ulcers or scars not thought to be due to trauma. Digital pitting scars are depressed areas at the tips as a result of ischemia, rather than trauma or exogenous causes.
Reduced forced vital capacity (FVC) percent predicted	According to local laboratory standards, <80% of predicted or lower cut point.
Interstitial lung disease or pulmonary fibrosis	Pulmonary fibrosis on HRCT or chest radiograph, most pronounced in the basilar portions of the lungs, or presence of ‘Velcro’ crackles on auscultation.
Persistent or recurrent gastro-esophageal reflux disease	By history, endoscopy or imaging.
Puffy fingers	Swollen 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.
Pulmonary arterial hypertension	Pulmonary arterial hypertension diagnosed by right heart catheterization.
Raynaud’s phenomenon	Self report or reported by a physician with at least a two-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.
Renal crisis	New onset of a systolic blood pressure (SBP) ≥ 140 mmHg and diastolic blood pressure (DBP) ≥ 90 mmHg; OR a rise in SBP ≥ 30 mmHg compared to usual and rise in DBP ≥ 20 mmHg compared to usual, AND at least 1 of these features: 1. Serum creatinine: increase of ≥ 50% above usual level; 2. Proteinuria: ≥ 2+ by dipstick confirmed by protein: creatine ratio > ULN; 3. Hematuria: ≥ 2+ by dipstick or > 10 RBCs/HPF (without menstruation); 4. Thrombocytopenia: < 100,000/mm3; 5. Hemolysis (fragmented RBC: by blood smear or increased reticulocyte count.
Scleroderma (skin thickening)	Skin thickening or hardening anywhere but not due to scarring after injury, trauma, etc.
Telangiectasias	Telangiectasias in a scleroderma like pattern are round and well demarcated and found on hands, lips, inside of the mouth, and/or large matt-like telangiectasias. Telangiectasias are visible macular dilated superficial blood vessels which collapse upon pressure and fill slowly when pressure is released; distinguishable from rapidly filling spider angiomas with central arteriole and from dilated superficial vessels.
Tendon or bursal friction rubs	One or more friction rubs detectable at places such as shoulders, olecranon bursae, wrists (flexor or extensors), fingers (flexor or extensor), knees, ankles (Achilles, peroneal, posterior tibial, or anterior tibial tendons).