Three-year trajectories of disability and fatigue in systemic sclerosis: a cohort study.

OBJECTIVES: Functional disability and fatigue are important consequences of systemic sclerosis (SSc), but little is known about their course over time. The aim of this study was to identify and characterise homogeneous subgroups with distinct 3-year trajectories of disability and fatigue, separately. METHODS: A 3-year cohort study including 215 patients with SSc was conducted. Functional disability was assessed using the Health Assessment Questionnaire-Disability Index (HAQ-DI). Fatigue was assessed using the SF-36 Vitality subscale. Longitudinal trajectories were identified using latent class growth analyses (LCGA). Baseline patient characteristics were compared across classes using multivariable logistic regression. RESULTS: Two disability classes were identified: a 'low' group (n=133) with low baseline HAQ-DI scores (intercept=0.48) and slight, statistically non-significant deterioration over time (slope=0.01), and a 'high' group (n=82) with high baseline HAQ-DI scores (intercept=1.63) and also slight, statistically non-significant deterioration over time (slope=0.01). Patients in the high disability group were more likely to be female, have higher fatigue, more helplessness, and less emotion-focused coping. Two fatigue classes were identified: an 'average' group (n=99) with average baseline Vitality scores (intercept=53.9) and slight, statistically non-significant deterioration over time (slope=-0.23), and a 'high' fatigue group (n=116) with low baseline Vitality scores (intercept=39.8) and also slight, but non-significant deterioration over time (slope=-0.15). Patients in the high fatigue group were more likely to be female, report more impact of lung involvement, and less acceptance. CONCLUSIONS: Functional disability and fatigue trajectories in SSc were relatively stable over a 3-year period, and differences in baseline scores, but not slopes, defined classes.

Content of non-pharmacological care for systemic sclerosis and educational needs of European health professionals: a EUSHNet survey.

OBJECTIVES: To describe the non-pharmacological care in systemic sclerosis (SSc) provided by European health professionals (HPs) including referrals, treatment targets, interventions, and educational needs. METHODS: In this observational study, European HPs working in SSc care were invited to complete an online survey through announcements by EUSTAR (European League Against Rheumatism (EULAR) Scleroderma Trials and Research) and FESCA (Federation of European Scleroderma Associations), the EULAR HPs' newsletter, websites of national patient and HP associations, and by personal invitation. RESULTS: In total, 56 HPs, from 14 different European countries and 7 different disciplines, responded to the survey. A total of 133 specific indications for referral were reported, 72% of which could be linked to the International Classification of Functioning, Disability and Health domain "body functions and structures". Of the 681 reported treatment targets 45% was related to "body functions and structures". In total, 105 different interventions were reported as being used to address these treatment targets. Almost all (98%) respondents reported having educational needs, with the topics of management of stiffness (67%), pain (60%), and impaired hand function (56%) being mentioned most frequently. CONCLUSIONS: Non-pharmacological care in SSc varies in Europe with respect to the content of interventions, reasons for referral, and treatment targets. Reasons for referral to HPs are not well-aligned to HPs subsequent treatment targets in SSc care suggesting suboptimal communication between physicians and HPs. The wide variations reported indicate a need to consolidate geographically disparate expertise within countries and to develop and improve standards of non-pharmacological care in SSc.

Effectiveness of Nonpharmacologic Interventions in Systemic Sclerosis: A Systematic Review.

OBJECTIVE: To systematically and comprehensively document the effectiveness of nonpharmacologic interventions on physical functioning and psychological well-being in patients with systemic sclerosis (SSc). METHODS: Multiple electronic databases were searched for studies on the effectiveness of nonpharmacologic interventions in SSc. Randomized clinical trials (RCTs), controlled clinical trials (CCTs), and observational designs (ODs) with ≥10 participants were included. Two reviewers independently assessed methodologic quality using the Downs and Black checklist. RESULTS: Twenty-three studies (9 RCTs, 4 CCTs, and 10 ODs) were included. Studies assessing comparable interventions were grouped, resulting in data for 16 different interventions. The total number of patients included per study ranged from 10 to 53. Seventeen different outcome domains were assessed, with hand function, limitations in activities, and quality of life being assessed most frequently. Three studies, all RCTs, were rated as high quality. These RCTs reported that 1) a multifaceted oral health intervention improves mouth hygiene, and additional orofacial exercises did not improve mouth opening, 2) a multidisciplinary team-care program improves limitations in activities, mouth opening, and hand grip strength, and 3) manual lymph drainage improves hand function, limitations in activities, and quality of life. CONCLUSION: The body of knowledge regarding nonpharmacologic care in SSc is very limited due to the wide variety in studied interventions and outcomes in the relatively uncommon but highly disabling disease. To structure and focus future research, an international consensus should be established to prioritize primary targets for nonpharmacologic treatment and the content of interventions and to agree on a core set of outcome measures.

Cognitive-behavioural therapy targeting fear of progression in an interdisciplinary care program: a case study in systemic sclerosis.

People living with chronic illness often report uncertainty about the future, fear of disease progression, fear of becoming physically disabled, and a reduced life expectancy as important sources of stress. However, little is known about psychological interventions targeting these concerns. The aim of this study is to illustrate an intervention to reduce emotional distress and concerns about the future in a patient with systemic sclerosis (SSc), a rare chronic rheumatic disease with serious consequences for most patients, and to present a preliminary report on its effectiveness using a single-case study design. Because of the complexity of symptoms and complaints due to SSc, the psychological intervention was embedded in an interdisciplinary care program also consisting of physical therapy, occupational therapy, and specialized nurse care.

The comparability of English, French and Dutch scores on the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F): an assessment of differential item functioning in patients with systemic sclerosis.

OBJECTIVE: The Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F) is commonly used to assess fatigue in rheumatic diseases, and has shown to discriminate better across levels of the fatigue spectrum than other commonly used measures. The aim of this study was to assess the cross-language measurement equivalence of the English, French, and Dutch versions of the FACIT-F in systemic sclerosis (SSc) patients. METHODS: The FACIT-F was completed by 871 English-speaking Canadian, 238 French-speaking Canadian and 230 Dutch SSc patients. Confirmatory factor analysis was used to assess the factor structure in the three samples. The Multiple-Indicator Multiple-Cause (MIMIC) model was utilized to assess differential item functioning (DIF), comparing English versus French and versus Dutch patient responses separately. RESULTS: A unidimensional factor model showed good fit in all samples. Comparing French versus English patients, statistically significant, but small-magnitude DIF was found for 3 of 13 items. French patients had 0.04 of a standard deviation (SD) lower latent fatigue scores than English patients and there was an increase of only 0.03 SD after accounting for DIF. For the Dutch versus English comparison, 4 items showed small, but statistically significant, DIF. Dutch patients had 0.20 SD lower latent fatigue scores than English patients. After correcting for DIF, there was a reduction of 0.16 SD in this difference. CONCLUSIONS: There was statistically significant DIF in several items, but the overall effect on fatigue scores was minimal. English, French and Dutch versions of the FACIT-F can be reasonably treated as having equivalent scoring metrics.

Health care use and patients' perceptions on quality of care in systemic sclerosis.

OBJECTIVES: To assess health care utilisation in Dutch patients with systemic sclerosis (SSc) and its associated factors. To evaluate patients' perspectives on quality of care and its association with health care use. METHODS: In a cross-sectional design, 198 Dutch patients with SSc completed an anonymous survey concerning health care utilisation, quality of care (CQ Index), and quality of life (SF-36). RESULTS: In the last 12 months, 95% of the patients had contact with at least one medical specialist and two-thirds contacted at least one health professional (HP). The median numbers of visits to medical specialists and HPs were 7 and 7.5, respectively. Having a partner and reduced physical health status (SF-36 role-physical) were significantly associated with more visits to medical specialists and HPs. The median numbers of disciplines contacted since the onset of SSc and in the last 12 months were 8 and 4, respectively. Patients with less fatigue (SF-36 vitality) and more pain (SF-36 bodily pain) contacted more disciplines. A higher number of disciplines involved in the care was significantly associated with less satisfaction with the coordination of care (r=-0.14, p=0.03). CONCLUSIONS: Health care utilisation in Dutch patients with SSc is substantial, as is reflected in the high number of visits and the number of disciplines. Patients' rating of care coordination was lower if more disciplines were involved in their care.