Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI) Item Score Musculoskeletal and skin Joint contracture defned as any desgree of contracture with the inability to reduce the joint to the † 2 anatomically neutral position in any small joint of the fngers.* Joint contracture defned as any desgree of contracture with the inability to reduce the joint to the † 2 anatomically neutral position in any in the large joints, specifcally elbows and knee.* Sicca symptoms defned as presence of dry eyes and/or dry mouth requiring treatment on a daily basis, for † 3 example, lubricant eye-drops, punctual plugs, saliva replacment.* Proximal muscle weakness on clinical examination defned as shoulder abduction and/or hip or knee 3 fexion less than 5/5 power (not due to contracture or pain) Calcinosis complicated by infection or requiring surgery. 4 Vascular Digital ulceration defned as loss of epithelialisation, of any degree, of the epidemis, the dermis and/ 2 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* Add 1 if digital amputation required (surgical or autoamputation). 1 Gastrointestinal Oesophageal dysmotility defned as distal dysphagia refractory to treatment, with differential diagnoses 1 (eg, oesophageal srricture or malignancy) excluded by endoscopy. Oesophageal stricture confrmed on testing such as endoscopy or barium swallow. 1 Symptoms of gastro-oesophageal refux disease (heart bum) refractory to treatmant (eg, proton pump 1 inhibitors) and confrmed on endoscopy.* Gastric antral vascular ectasia confrmed on endoscopy. 2 Pseudo-obstruction with symptoms such as vomiting or constipation, with dilatation of the small and/or 3 large bowel on imaging. Low body mass index of <18.5 kg/m2 or weigt loss of 10% in the lass 12 months. 2 Respiratory Moderate to severe interstitial lung disease >20% extent on HRCT of the chest. 2 Add 4 points if forced vital capacity <70% lung function tests (not due to respiratory muscle 4 weakness).* Dependence on home oxygen. 5 Cordivascular Pulmonary arterial hypertension (defned as mean pulmonary arterial pressure ≥ 25mm Hg at rest and 2 pulmonary arterial wedge pressure ≤15mm Hg on right heart catheterisation) Add 5 if moderate to severe right ventricular dysfunction noted on echocardiogrephy report based on 5 assessment of any measure of RV function by experienced cardiologist. Myocardial disease attributable to SSc based on a constellation of clinical features and supportive investigations, for example, syncope secondary to conduction abnormality, arrythmia requiring defbrillator, 3 hearblock requiring permanent pacemaker or ablation, systolic or diastolic dysfunction or TTE. Presence of moderate to large pericardial effusion equivalent or greater than 1cm on TTE* 1 Renal Ferdowsi N, Huq M, Stevens W, et al. Ann Rheum Dis 2019;78:807–816. כלי מחקרי יחסית חדש להערכת נזק מצטבר ובלתי הפיך בסקלרודרמה. חלק מהמדדים נכללים אם היו בששת החודשים האחרונים. המדד נמצא בקורלציה לפגיעה במערכות השונות במחלה, ובקורלציה לתמותה. 33
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