| Agreement from round 3 Median (25th–75th percentile) | ||
| Skin: | ||
| 1 | Modified Rodnan skin score (range 0–51) | 9 (9–9) |
| 2 | Visual analogue scale (VAS)/Likert score of patient’s global assessment for skin activity* | 7 (5.5–9) |
| 3 | VAS/Likert score of doctor’s global assessment for skin activity* | 7 (6–9) |
| 4 | Durometer | 7 (6–8) |
| Musculoskeletal: | ||
| 1 | Tender joint count | 6 (5–8) |
| 2 | Tendon friction rubs assessed by the doctor* | 8 (7–9) |
| 3 | Serum creatinine phosphokinase, aldolase | 7 (6–9) |
| Cardiac: | ||
| 1 | (1)Cardiac echocardiogram with Doppler† | 9 (8–9) |
| 2 | (2)Right heart catheterisation‡ | 8 (6–9) |
| 3 | 6-min walk test‡ | 8 (7–9) |
| 4 | Borg dyspnoea instrument‡ | 7 (6–9) |
| Pulmonary: | ||
| 1 | (3)Pulmonary function testing | 9 (9–9) |
| 2 | (A)Validated measure of dyspnoea | 8 (7–9) |
| 3 | Breathing VAS from the Scleroderma Health Assessment Questionnaire (S-HAQ)23 | 7 (6–9) |
| 4 | High resolution computer tomography (HRCT) of the lungs: quantifiable scale‡ | 9 (7–9) |
| Renal: | ||
| 1 | Calculated creatinine clearance based on serum creatinine (Cockroft–Gault or Modification of Diet in Renal Disease (MDRD) formula) | 9 (7–9) |
| 2 | Pre-defined renal crisis (presence or absence) | 9 (8–9) |
| Gastrointestinal: | ||
| 1 | Body mass index (BMI) | 7 (6–9) |
| 4 | Validated gastrointestinal (GI) tract VAS scale (part of S-HAQ)23 or other SSc-validated GI questionnaire | 8 (7–9) |
| Health-related quality of life and function: | ||
| 1 | Health Assessment Questionnaire-Disability Index (HAQ-DI)24 | 9 (7–9) |
| 2 | VAS pain from the HAQ-DI24 | 7 (6–9) |
| 3 | Short form-36 (SF-36) version 225 | 8 (7–9) |
| Global health: | ||
| 1 | VAS/Likert patient global severity | 8 (6.5–9) |
| 2 | VAS/Likert doctor global severity | 8 (6–9) |
| 3 | (B)Scleroderma-related health transition by patient | 7 (6–9) |
| 4 | (C)Scleroderma-related health transition by doctor¶ | 7 (5–8) |
| Raynaud phenomenon: | ||
| 1 | Raynaud condition score26 | 8 (7–9) |
| 2 | VAS Raynaud (part of S-HAQ)23 | 8 (6.5–9) |
| Digital ulcers: | ||
| 1 | Active digital tip ulcer count on the volar surface | 9 (7–9) |
| 2 | VAS digital ulcer (part of S-HAQ)23 | 8 (6–9) |
| Biomarkers: | ||
| 1 | Acute 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.