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.

All four disease activity composite indices were found to be valid measures for assessing arthritis in SSc. DAS28-ESR showed the best performance regarding reliability and construct validity.

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.