Effectiveness of a multidisciplinary team care programme in comparison with usual care for patients with scleroderma

1. 6-minute walk test (metre)
Validated in patients with rheumatic, cardiac and lung disease and is extensively used in trials in patients with scleroderma aiming at improvement of exercise tolerance. Assuming a 30% improvement in the intervention group and 0% in the control group, á=0.05 and â =0.20 (power of 0.80), 22 patients per group would be needed to detect this difference (N = 7.85 x 0.15(0.85) x 2 / (0.3)2)
2. The Physical Component Summary scale (Phys-SS) of the Short Form-36 (SF-36)
A generic, multi-dimensional quality of life questionnaire. Phys-SS includes physical functioning, role-physical, bodily pain, vitality and general health. Assuming an standard deviation (SD) of the Phys-SS baseline score of 11, an improvement of 10 points in the intervention and 0 in the control group, ≥ 0.05 and ≤ 0.20, 19 patients per group would be needed.
3. The Scleroderma-Health Assessment Questionnaire (SHAQ)
Assesses difficulty with daily activities (20 items) and interference of scleroderma symptoms with activities (visual analogue scale [VAS] 0-10 cm for Raynaud phenomenon, finger ulcers, lung problems, gastrointestinal problems, general disease related problems, and pain). Assuming a 22% improvement in the intervention group and 0% in the control group, ≥ 0.05 and power of 0.80, 28 patients per group would be needed to detect this difference (N = 7.85 x 0.11(0.78) x 2 / (0.22)2).

Based on an average of these calculations we aimed to enrol 50 patients in the study.

Baseline characteristics assessed at baseline only. All other assessments carried out at baseline, 12 and 24 weeks. Assessments are done by a trained assessor, who is blinded for the treatment condition.

1. The Hand Mobility in Scleroderma (HAMIS) test
Consisting of 9 items graded on a scale of 0-3, the final score ranges from 0 (normal function) to 27 (severe immobility). It was found to be a reliable instrument in evaluation of hand function in SSc and longitudinal assessment of hand mobility in early SSc.
2. Grip strength (kg)
Measured with a Jamar dynamometer. After testing twice, the highest score was registered. An average value of the grip strength of the left and right hand was computed.
3.Maximal Mouth Opening (MMO)
Measured with a digital calliper as the maximal interdentally distance. Measurement of the maximal mouth opening was used in several studies evaluating mouth function in SSc.
4. Maximal oxygen uptake (VO2max)
A standard exercise test on an electronically braked cycle ergo meter performed according to the American Thoracic Society /American College of Chest Physicians (ATS/ACCP) Statement on Cardiopulmonary Exercise Testing. At baseline (forehead) pulse oximetry, blood pressure, heart rate and gas exchange were recorded. Pulse oximetry and heart rate were monitored during 1 min of rest, 2 min of unloaded cycling at 60 rpm followed by an increasing load to maximum tolerance, and 3 min of recovery. Each exercise test was supervised by a pulmonary physician. Apart from the VO2 max, maximal rate (Watt), heart rate and ventilation at maximal tolerated work rate were monitored.
5. Checklist Individual Strength-20 (CIS-20)
A Dutch generic 20-item self-report instrument that measures 4 dimensions of fatigue: fatigue, concentration, impaired motivation and impaired activity on a seven-point Likert scale. Higher scores indicate a higher degree of fatigue, more concentration problems, reduced motivation, and less activity. The CIS developed for patients with chronic fatigue syndrome, and has good psychometric properties.

Baseline characteristics assessed at baseline only. All other assessments carried out at baseline, 12 and 24 weeks. Assessments are done by a trained assessor, who is blinded for the treatment condition.