The construct validity of the EScSG activity index is good, though the lung-related disease activity may not be sufficiently represented. Further validation steps may be required for both the EScSG and our 12-point activity index.

Background Musculoskeletal manifestations including arthralgia/arthritis, tendon friction rubs, joint contractures, digital tuft resorption, calcinosis and muscle weakness are the major causes of disability in systemic sclerosis (SSc). Joint involvement has been reported to occur in up to 46% to 97% of SSc patients. Recently complex individualized rehabilitation programs seem to be effective. The optimal therapy for SSc and musculoskeletal symptoms is still not known, and there are only a few joint-observational investigations longer than one year available in the literature. Objectives To observe the effects of home exercise on changes in range of motion (ROM) and contracture development in a three-year follow-up study in patients with systemic sclerosis. Methods One hundred and thirty-one consecutive patients, 119 females and 12 males, 41 with diffuse cutaneous SSc (dcSSc), 90 with limited cutaneous SSc (lcSSc) were evaluated at baseline, with 115 patients re-evaluated at 1 and 3 years. Mean age was 55.9±11.6 years (±SD) with a mean disease duration of 8.1±7.2 years. ROM, Modified Rodnan Skin Score (MRSS), Health Assessment Questionnaire (HAQ-DI), Hand Anatomic Index (HAI) and clinical characteristics were recorded. Additionally, every patient at our centre receives instruction for home exercises of hands, mouth and large joints. This cycle is repeated at least every 6 months. To determine differences between sub-groups Mann-Whitney-U test were performed. Spearman’s Rank Correlation Coefficient was calculated to assess parametric correlation. Results Limitation in ROM greater than 25% was considered “contracture” and greater than 50% as “severe contracture”. At baseline, MCP II and III were the most commonly affected joints, in 72-77% of patients. Wrist flexion-extension was impaired in 69-75%, shoulder flexion-extension in 49-50%, PIP II-III flexion-extension in 34-43%, wrist adduction-abduction in 18-22%, knee flexion-extension in 15-17%, shoulder adduction-abduction in 13-15%, rotation in 9-11%, ankle extension-flexion, hip rotation and flexion-extension in 7-8%, abduction in 1-2%. Over a 3-year-follow up period, differences favouring the non-dominant hand in ROM were statistically significant while there was no difference in laterality of the large joints. Throughout the follow-up only the small joints (MCPII, III and PIP III) of the hands, the results of the 10-meter walk test and the HAI showed significant improvement. However the large joints, revealed no significant difference. The values of the functional indexes (HAQ-DI and DASH) showed significant improvement (p<0,05) only at the end of the first year in early dcSSc-group. The number of severe contractures of the upper extremities positively correlated with ESR, C-reactive protein, HAQ-DI and the 10-meter walk test and negatively with forced vital capacity (FVC) at baseline and 3-year-follow up. Conclusions A simple “routine” stretching exercises performed at home may be beneficial for small joint function, but does not have a noticeable effect o...

We validated the skin thickness domain of the questionnaire, except the responsiveness. Because of the low mean MRSS of patients in this investigation, the patient self-assessment validation procedure should be repeated with cases exhibiting more extensive skin involvement. Patient-reported and examiner-measured tethering may not be used as independent instruments of skin involvement. The skin thinness domain may contain valuable independent information, therefore it also merits further investigation.

Background Joint contractures as well as symptoms of cardiopulmonary involvement restrict the physical capacity of patients with systemic sclerosis (SSc). The six minute walk test (6MWT) is widely used in the assessment of physical capacity and to evaluate treatment efficacy. However, it is unclear how much the joint function of the patients influences the diagnostic value of the walking tests. Objectives We performed a prospective, cross sectional, single center study to assess the 6MWT, the 10-meter walk test (10meterWT) in the context of the joint contractures. The relationship between the extent of joint-contractures to the major internal organ involvements including different forms of cardiopulmonary involvement was also examined. Methods Ninety-six consecutive patients, 84 females and 12 males, 32 with diffuse cutaneous SSc (dcSSc), 64 with limited cutaneous SSc (lcSSc) were enrolled during their routine yearly scheduled check-up. Patients’ mean age was 56.2±12.7 years (±SD) with a mean disease duration of 8.8±7.5 years. The protocol included physical examination, echocardiography and 6MWT. Additionally, Health Assessment Questionnaire Disability Index (HAQ DI), and European SSc activity index were determine and the 10meterWT was also performed. Limitation in range of motion of the joints greater than 25% was considered “contracture”, and the number of contractures of each extremity was recorded. Results The mean distance of the 6MWT was 363±66 m. Neither the number of contractures of the lower extremities, nor the number of the whole body’s all contractures showed significant correlation with the 6-minute walk distance in SSc. HAQ DI (p<0.001) and age (p<0.05) were independent predictors of the results of both walking tests. Predictors of 6MWT included the right ventricular systolic pressure, and the number of digital ulcers, while a trend for the presence of left heart failure was also found (p=0.057). Further significant determinants of the 10-meterWT were the extent of the joint restrictions of the lower extremities (p<0.01), and the disease activity index (p<0.05). Conclusions The number of joint contractures does not influence significantly the applicability of 6MWT in SSc. The relation to the right ventricular systolic pressure underlines its applicability in characterizing patients with left or right heart dysfunction. The 10MWT where the patients are expected to walk only 10 meters as opposed to the hundreds of meters covered in the 6MWT, is the more sensitive indicator of the joint involvement. Disclosure of Interest None Declared