Item | Mortality coeff | Morbidity coeff | Combined coeff | Weighted score | |
Musculoskeletal and skin | |||||
1 | Joint contracture defined as any degree of contracture with the inability to reduce the joint to the anatomically neutral position in any small joint of the fingers. | −0.35 | 0.49 | 0.49 | 2 |
2 | Joint contracture defined as any degree of contracture with the inability to reduce the joint to the anatomically neutral position in the large joints, specifically elbows and knees. | −0.35 | 0.49 | 0.49 | 2 |
3 | Sicca symptoms defined as presence of patient-reported dry eyes and/or dry mouth requiring treatment on a daily basis, for example, lubricant eye-drops, punctual plugs, saliva replacement. | −0.10 | 0.75 | 0.75 | 3 |
4 | Proximal muscle weakness on clinical examination defined as shoulder abduction and/or hip or knee flexion less than 5/5 power (not due to contracture or pain). | 0.60 | 0.92 | 0.76 | 3 |
5 | Calcinosis complicated by infection or requiring surgery. | −0.21 | 1.08 | 1.08 | 4 |
Vascular | |||||
6 | Digital ulceration defined as loss of epithelialisation, of any degree, of the epidermis, the dermis and/or the subcutaneous tissue, distal to or at the proximal interphalangeal joint of the hands or feet not thought to be due to trauma and refractory to therapy. | 0.79 | 0.37 | 0.58 | 2 |
7 | Digital amputation (surgical or autoamputation). | 0.96 | 1.09 | 1.03 | 3 |
Gastrointestinal | |||||
8 | Oesophageal dysmotility defined as distal dysphagia refractory to treatment with differential diagnoses (eg, oesophageal stricture or malignancy) excluded by endoscopy. | −0.21 | 0.12 | 0.12 | 1 |
9 | Pseudo-obstruction with symptoms such as vomiting or constipation, with dilatation of the small and/or large bowel on imaging. | 0.93 | −0.82 | 0.93 | 3 |
10 | Low body mass index of <18.5 kg/m2 or weight loss of >10% in the last 12 months. | 0.68 | 0.74 | 0.71 | 2 |
11 | Gastric antral vascular ectasia confirmed on endoscopy. | 0.67 | 0.32 | 0.50 | 2 |
12 | Oesophageal stricture confirmed on testing such as endoscopy or barium swallow. | 0.15 | 0.20 | 0.18 | 1 |
13 | Symptoms of gastro-oesophageal reflux disease (heart burn) refractory to treatment and confirmed on endoscopy. | 0.03 | 0.71 | 0.37 | 1 |
Respiratory | |||||
14 | Moderate to severe interstitial lung disease with >20% extent on HRCT of the chest. | 0.19 | 0.97 | 0.58 | 2 |
15 | Moderate to severe interstitial lung disease with >20% extent on HRCT of the chest and forced vital capacity <70% on lung function tests. | 1.11 | 2.47 | 1.79 | 6 |
16 | Dependence on home oxygen. | 0.88 | 1.99 | 1.44 | 5 |
Cardiovascular | |||||
17 | Pulmonary arterial hypertension (defined as mean pulmonary arterial pressure >25 mm Hg at rest and pulmonary arterial wedge pressure <15 mm Hg on right heart catheterisation). | 0.75 | 0.59 | 0.67 | 2 |
18 | Moderate to severe right ventricular dysfunction noted on echocardiography report based on assessment of any measure of RV function by experienced cardiologist. | 1.81 | 2.49 | 2.15 | 7 |
19 | Myocardial disease attributable to SSc based on a constellation of clinical features and supportive investigations, for example, syncope secondary to conduction abnormality, arrhythmia requiring defibrillator, heart block requiring permanent pacemaker or ablation, systolic or diastolic dysfunction on TTE. | 0.85 | 0.77 | 0.81 | 3 |
20 | Presence of moderate to large pericardial effusion equivalent to greater than 1 cm on TTE. | 0.45 | 0.09 | 0.27 | 1 |
Renal | |||||
21 | History of scleroderma renal crisis (SRC), either hypertensive or normotensive, as defined by the International Scleroderma Renal Crisis Study Investigators.45 | 0.46 | 1.54 | 1.00 | 3 |
22 | History of SRC or other SSc-related kidney disease and persistent renal impairment with estimated glomerular filtration rate <45 mL/min/1.73 m2. | 0.66 | 1.76 | 1.21 | 4 |
23 | SRC with stage 5 renal impairment defined as need for renal replacement therapy. | 1.08 | 2.16 | 1.62 | 5 |
Coeff, coefficient;HRCT, high-resolution CT;RV, right ventricular;SCTC-DI, Scleroderma Clinical Trials Consortium-Damage Index; SSc, systemic sclerosis; TTE, transthoracic echocardiogram.