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Systemic sclerosis (SSc), or scleroderma, is a chronic multisystem autoimmune disorder characterised by thickening and fibrosis of the skin and by the involvement of internal organs such as the lungs, kidneys, gastrointestinal tract, and heart. Because there is no cure, feasibly-implemented and easily accessible evidence-based interventions to improve health-related quality of life (HRQoL) are needed. Due to a lack of evidence, however, specific recommendations have not been made regarding non-pharmacological interventions (e.g. behavioural/psychological, educational, physical/occupational therapy) to improve HRQoL in SSc. The Scleroderma Patient-centred Intervention Network (SPIN) was recently organised to address this gap. SPIN is comprised of patient representatives, clinicians, and researchers from Canada, the USA, and Europe. The goal of SPIN, as described in this article, is to develop, test, and disseminate a set of accessible interventions designed to complement standard care in order to improve HRQoL outcomes in SSc.

Objective: To identify the clinical characteristics of systemic sclerosis (SSc) that best correlate with the health-related quality of life (HRQOL) of patients with SSc, using the World Health Organization Disability Assessment Schedule II (WHODAS II) as the measure of HRQOL., Methods: A cross-sectional, multicenter study of 337 patients from the Canadian Scleroderma Research Group Registry was conducted. Patients were assessed through detailed clinical histories, medical examination, and the WHODAS II. Hierarchical multiple linear regression was used to assess the relationship between selected clinical variables and HRQOL., Results: The mean WHODAS II score was 23.7 (range 0-100), with the greatest impairments seen in the subscales measuring life activities, mobility, and participation in society. In multivariate analysis, significant predictors of the WHODAS II were skin scores, shortness of breath, number of gastrointestinal problems, fatigue, pain, and depression. The final model explained 61% of the variance in the WHODAS II scores., Conclusion: The clinical characteristics identified in this study as significant correlates of HRQOL in SSc should each be targets of intervention in order to improve the HRQOL of patients with this disease.

Objective: To determine the validity of the World Health Organization Disability Assessment Schedule II (WHODAS II) in systemic sclerosis (SSc)., Methods: Patients enrolled in the Canadian Scleroderma Research Group registry participated in a standardized evaluation and completed the WHODAS II. Criterion validity was assessed by comparing the WHODAS II with the Medical Outcomes Study Short Form 36 (SF-36), construct validity was assessed by examining how it relates to common measures of outcome in SSc, and discriminative validity was assessed by examining how it distinguishes patients with more severe disease from those with less severe disease., Results: A total of 402 patients with SSc were included (mean +/- SD age 55 +/- 13 years, 87% women, mean +/- SD disease duration 11 +/- 9 years). The mean +/- SD WHODAS II score was 24.6 +/- 17.4, and the greatest impairments were in life activities and mobility. There were moderate to good correlations between the WHODAS II and the SF-36 Physical Component Summary score (r = -0.44), the SF-36 Mental Component Summary score (r = -0.41), and measures of function (r = 0.54), depression (r = 0.44), pain (r = 0.40), and fatigue (r = -0.49, P < 0.0001 for all). The WHODAS II was able to consistently distinguish patients with milder disease from those with more severe disease., Conclusion: The WHODAS II had good psychometric properties in patients with SSc and should be considered a valid measure of health-related quality of life in SSc.