Table 1 Core set items selected for 11 domains
Agreement from round 3 Median (25th–75th percentile)
1Modified Rodnan skin score (range 0–51)9 (9–9)
2Visual analogue scale (VAS)/Likert score of patient’s global assessment for skin activity*7 (5.5–9)
3VAS/Likert score of doctor’s global assessment for skin activity*7 (6–9)
4Durometer7 (6–8)
1Tender joint count6 (5–8)
2Tendon friction rubs assessed by the doctor*8 (7–9)
3Serum creatinine phosphokinase, aldolase7 (6–9)
1(1)Cardiac echocardiogram with Doppler†9 (8–9)
2(2)Right heart catheterisation‡8 (6–9)
36-min walk test‡8 (7–9)
4Borg dyspnoea instrument‡7 (6–9)
1(3)Pulmonary function testing9 (9–9)
2(A)Validated measure of dyspnoea8 (7–9)
3Breathing VAS from the Scleroderma Health Assessment Questionnaire (S-HAQ)237 (6–9)
4High resolution computer tomography (HRCT) of the lungs: quantifiable scale‡9 (7–9)
1Calculated creatinine clearance based on serum creatinine (Cockroft–Gault or Modification of Diet in Renal Disease (MDRD) formula)9 (7–9)
2Pre-defined renal crisis (presence or absence)9 (8–9)
1Body mass index (BMI)7 (6–9)
4Validated gastrointestinal (GI) tract VAS scale (part of S-HAQ)23 or other SSc-validated GI questionnaire8 (7–9)
Health-related quality of life and function:
1Health Assessment Questionnaire-Disability Index (HAQ-DI)249 (7–9)
2VAS pain from the HAQ-DI247 (6–9)
3Short form-36 (SF-36) version 2258 (7–9)
Global health:
1VAS/Likert patient global severity8 (6.5–9)
2VAS/Likert doctor global severity8 (6–9)
3(B)Scleroderma-related health transition by patient7 (6–9)
4(C)Scleroderma-related health transition by doctor¶7 (5–8)
Raynaud phenomenon:
1Raynaud condition score268 (7–9)
2VAS Raynaud (part of S-HAQ)238 (6.5–9)
Digital ulcers:
1Active digital tip ulcer count on the volar surface9 (7–9)
2VAS digital ulcer (part of S-HAQ)238 (6–9)
1Acute phase reactant(s): erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP)8 (6–9)
  • Parameters to be measured for the items in the core set:

  • (1)Echocardiogram with Doppler: measure pulmonary artery systolic pressure, left and right ventricular systolic and diastolic parameters, pericardial effusion, and chamber size.

  • (2)Right heart catheterisation: pulmonary artery pressure, pulmonary vascular resistance, wedge pressure, cardiac output/cardiac index.

  • (3)Pulmonary function test: forced vital capacity (FVC), diffusion capacity (DLCO), total lung capacity (TLC).

  • (A)Validated dyspnoea questionnaires, eg, University of California San Diego (UCSD) Dyspnoea Questionnaire, St. George Respiratory Questionnaire, Mahler Dyspnoea Index.

  • (B)Scleroderma-related health transition by patient; compared to 1 year ago, how do you rate your overall scleroderma: much better, a little better, no change, a little worse, much worse?

  • (C)Scleroderma-related health transition by doctor; compared to 1 year ago, how do you rate your patient’s overall scleroderma: much better, a little better, no change, a little worse, much worse?

  • *Items were based on Steering Committee and Scleroderma Clinical Trials Consortium (SCTC) consensus despite lack of full validation.

  • †Standardised central reading mechanism strongly encouraged, ‡if relevant to the study.