Validity and reliability of the Swedish version of the Self-Efficacy for Managing Chronic Disease scale for individuals with systemic sclerosis.

OBJECTIVE: To investigate aspects of validity and reliability of the Swedish version of the Self-Efficacy for Managing Chronic Disease (SEMCD-Swe) scale in systemic sclerosis (SSc). METHOD: A forward-backward translation procedure was used. Content validity was assessed through interviews with 11 people with SSc and 10 healthcare professionals. Construct validity, internal consistency, test-retest reliability, and floor and ceiling effects were evaluated in 104 SSc patients. RESULTS: The content validity of the SEMCD-Swe was interpreted as satisfactory, but some adjustments were made to increase the understanding. Confirmatory factor analysis supported a single-factor structure. Moderate to strong correlations between the SEMCD-Swe and Scleroderma Health Assessment Questionnaire; Multidimensional Assessment of Fatigue; Patient Health Questionnaire-8 (rs = -0.4 to -0.7), and RAND-36 subscales (rs = 0.5 to 0.7) were found. Weak correlations were found between SEMCD-Swe and modified Rodnan skin score; and disease severity of peripheral vascular and lung (rs = -0.1 to -0.2) and kidney (rs = 0.1) systems (Medsger severity scale). Cronbach's alpha was sufficient (0.85) and corrected item-to-total correlations were good (≥ 0.50). The intraclass correlation coefficient for the total score was sufficient (0.82). No floor or ceiling effects were found. CONCLUSION: Support for construct validity was indicated, as the SEMCD-Swe in SSc show a single-factor structure and is more strongly associated with pain, fatigue, depressive symptoms, interferences with daily activities, disability, and quality of life than with disease severity. Our results also indicate support for content validity and reliability. However, the responsiveness of the SEMCD-Swe needs to be tested.

Raynaud's phenomenon and its impact on activities in daily life during one year of follow-up in early systemic sclerosis.

OBJECTIVE: To investigate Raynaud's phenomenon (RP) and its impact on daily life activities during 1 year of follow-up in early systemic sclerosis (SSc). METHOD: Fourteen SSc patients with a median disease duration of 2 years were enrolled in the study. Every 7 weeks the patients completed a 7 day diary documenting the frequency and duration of RP attacks, the activity causing the attack, and how they handled the attack. The patients also recorded in the diary daily self-assessments of the difficulties with RP, using a 0-10 ordinal scale according to the Raynaud's Condition Score. RESULTS: Ninety-eight RP weekly diaries were analysed. The median number of RP attacks varied between six and nine per week, and the median score reflecting the difficulty associated with the attacks varied between 2.0 and 2.9. No difference was found in the number of attacks or the difficulties associated with them between winter, spring, and autumn. Fewer attacks and less difficulty were reported in August than in any of the other documented weeks (p < 0.05). In all diaries, all patients reported RP attacks associated with domestic activities. The use of heating devices varied during the follow-up. In February, all patients except one used such devices, while about half of the group used devices during the rest of the year. CONCLUSIONS: Difficulties resulting from RP are present and disabling all year round, which underscore the importance of intense vasoactive therapy and non-pharmacological strategies throughout the year.

Personal factors in systemic sclerosis and their coverage by patient-reported outcome measures. A multicentre European qualitative study and literature review.

BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease where thickening of the skin can lead to reduced body function and limitations in activities. Severe forms can also affect and seriously damage inner organs. Patient-centred rehabilitation emphasises considerations of patients' background, experience and behavior which highlights the need to know if patient-reported outcome measures (PROMs) include such personal factors. AIM: To identify and describe personal factors in the experiences of functioning and health of persons with SSc and to examine if and to what extent PROMs in SSc research cover these factors. DESIGN: Data from a qualitative study with focus group interviews were analysed. PROMs in SSc research were identified in a literature review between 2008-2013. SETTING: Participants were recruited from outpatient clinics at rheumatology department. POPULATION: Sixty-three patients with SSc from four European countries participated. METHODS: Data from interviews were analysed using a structure of personal factors developed by Geyh et al. Identified PROMs were analysed and linked to main concepts, related to the personal factors, found in the interview data. RESULTS: Nineteen main concepts were related to the area "patterns of experience and behaviour" in the personal factor structure, 16 to "thoughts and beliefs", nine to "feelings", one to "motives" and one to "personal history and biography", respectively. Among the 35 PROMs identified, 15 did not cover any of the identified concepts. Concepts within the area "feelings" were mostly covered by the PROMs. Five of the PROMs covered "patterns of experience and behaviour", while "motives" and "personal history and biography" were not covered at all. Four of the identified PROMs covered concepts within the areas "feelings", "thoughts and beliefs" and "patterns of experience and behaviour" in the same instrument. The Illness Cognition Questionnaire and Illness Behaviour Questionnaire were such PROMs. CONCLUSION: Patterns of experience and behaviour had the highest number of concepts related to personal factors, but few of the PROMs in SSc research covered these factors. Only a few PROMs covered several personal factors areas in the same instrument. CLINICAL REHABILITATION IMPACT: The results would be of value when developing core sets for outcome measurements in SSc.

Hand function and performance of daily activities in systemic lupus erythematosus: a clinical study.

This clinical study was performed to investigate hand problems in individuals with systemic lupus erythematosus (SLE) in comparison with healthy controls, and to explore problems in the performance of daily activities related to these hand problems, in order to objectify findings from a previous mail survey. We also investigated whether a simple hand test could detect hand problems in SLE. All individuals, 71 with SLE and 71 healthy controls, were examined for manifestations in body structures and body functions of the hands with a study-specific protocol. The simple hand test was performed by all the individuals and the arthritis impact measurement scale (AIMS 2) questionnaire was completed by the SLE individuals. In the SLE group, 58% had some kind of difficulty in the simple hand test, compared with 8% in the control group. Fifty percent of the SLE individuals experienced problems in performing daily activities due to hand deficits. Pain in the hands, reduced strength and dexterity, Raynaud's phenomenon and trigger finger were the most prominent body functions affecting the performance of daily activities. Deficits in hand function are common in SLE and affect the performance of daily activities. The simple hand test may be a useful tool in detecting hand problems.

Psychometric properties of the Swedish version of the Scleroderma Health Assessment Questionnaire and the Cochin Hand Function Scale in patients with systemic sclerosis.

OBJECTIVES: To translate the visual analogue scales (VAS) in the Scleroderma Health Assessment Questionnaire (SSc HAQ) and the Cochin Hand Function Scale (CHFS) and to examine the reliability and validity of the Swedish versions of the instruments. METHOD: The reproducibility, internal consistency, acceptability, and validity of the instruments were evaluated. Eighty-three consecutive patients participated in the evaluation of the SSc HAQ and 56 in the CHFS. Sixty-six per cent fulfilled the criteria for limited systemic sclerosis (lcSSc) and 29% for diffuse systemic sclerosis (dcSSc). The patients were assessed regarding disease parameters, hand involvement, and quality of life, the latter using the 36-item short form health survey (SF-36). RESULTS: The reproducibility in the HAQ Disability Index (HAQ-DI), the VAS of pulmonary, digital ulcer, and overall disease severity, and in the CHFS was good (intra-class correlation coefficients, ICCs ≥ 0.75). The internal consistency was high in the HAQ-DI and the CHFS but lower in the VAS. The HAQ-DI showed higher correlations coefficients with physical-related scores in the SF-36 (rs = -0.600) than with mental-related dimensions (rs = -0.235). All VAS showed significant correlation with the item for general health (p < 0.05). The CHFS showed high correlation to hand-related items in the HAQ (rs = 0.858) and moderate correlation to the physical summary score in SF-36 (rs = -0.521). The instruments could not discriminate between lcSSc and dcSSc, although significant correlations between the CHFS and hand involvement (p < 0.05) indicate the ability of the CHFS to discriminate between mild and severe hand involvement. CONCLUSIONS: The Swedish version of the SSc HAQ and the CHFS meet the requirements of reproducibility and concurrent validity. More studies are needed to examine the capacity of these instruments to discriminate between disease severities.

A longitudinal follow-up of hand involvement and activities of daily living in early systemic sclerosis.

OBJECTIVE: To investigate the development of hand involvement and activities of daily life (ADL) in early systemic sclerosis (SSc), and to examine the usefulness of the Hand Mobility in Scleroderma (HAMIS) test in a longitudinal study. METHODS: Forty-three patients with disease duration no longer than 3 years and at least 4 years of follow-up formed the study group. Based on skin involvement, 31 patients were classified as limited cutaneous SSc (lcSSc) and 12 as diffuse cutaneous SSc (dcSSc). The patients were assessed regarding hand function [mobility (HAMIS), grip force (Grippit), and self-assessed hand function visual analogue scale (VAS)], vascular involvement [Raynaud's phenomenon (RP)], skin involvement [modified Rodnan skin score (mRss)], and ADL capacity [the scleroderma Functional Score (FS)]. RESULTS: Hand mobility and ADL capacity were in general good and did not change significantly. However, at the individual level 72% of the patients showed a change in HAMIS score. Grip force and perceived hand function were moderately impaired at baseline and during the follow-up. Skin involvement in the hand/arm improved significantly (p<0.001). During the observation period, the hand/arm mRss and HAMIS score changed in parallel (r(s) = 0.58, p<0.001). ADL capacity correlated significantly with grip force, self-assessed hand function, and RP at baseline, and also with HAMIS at follow-up. CONCLUSIONS: In this study hand mobility and ADL capacity were maintained during the first years of SSc. HAMIS was found to be a feasible test for longitudinal assessment of hand mobility in SSc.

Working ability in relation to disease severity, everyday occupations and well-being in women with limited systemic sclerosis.

OBJECTIVE: To investigate how women with SSc and varying degrees of working ability differed regarding disease severity, everyday occupations and well-being. Working ability was operationalized according to the degree of sick leave. METHODS: Forty-four women of working age with lcSSc were assessed regarding sociodemographic characteristics, disease severity including organ manifestation, perceived physical symptoms, hand function, and satisfaction with everyday occupations, self-rated health and well-being. RESULTS: The subjects formed three groups with regard to reduction in working capacity. Twenty-one women (48%) had no sick leave, 15 women (34%) were on partial sick leave and eight women (18%) were temporarily on full-time sick leave or had a full disability pension. There were no statistically significant differences concerning sociodemographics between the groups. Women without sick leave had less physically demanding jobs (P = 0.026), and the hypothesis that working ability reflects lower disease severity was confirmed regarding dexterity grip force and perceived fatigue and breathlessness (P < 0.05). Greater working ability was associated with better capacity to perform activities of daily life (P < 0.01), greater satisfaction with occupations (P < 0.01), better well-being (P < 0.001) and better health (P < 0.001). CONCLUSIONS: Fifty per cent of the women were restricted in their working ability; the lower the working ability, the lower their perceived well-being. This emphasizes the need for further research into the factors that promote working ability and the development of suitable methods to improve working ability.

Hand function tests are important and sensitive tools for assessment of treatment response in patients with rheumatoid arthritis.

OBJECTIVES: To assess the usefulness of hand function measurements in a study of treatment effects of tumour necrosis factor (TNF) blockers and to define the relationship between different hand function tests and also relate hand function to general disability and disease activity. METHODS: The study group consisted of 49 patients with established rheumatoid arthritis (RA) who were followed for 1 year while on TNF inhibitors. Evaluation of hand function included Signals of Functional Impairment (SOFI), grip and pinch grip force, and the Grip Ability Test (GAT). General disability was assessed by the Health Assessment Questionnaire (HAQ) and disease activity by the 28-joint Disease Activity Score (DAS28). The standardized mean response (SMR) method was used to evaluate sensitivity to change for all hand tests using DAS28 and HAQ as external indicators of change. RESULTS: HAQ, DAS28, grip and pinch grip force, and GAT showed a highly significant improvement over time (p<0.001). The improvement in SOFI was also significant (p<0.01). The correlations between the different hand tests varied between 0.45 and 0.72. All hand function tests were significantly related to HAQ but showed only weak correlations to DAS28. SOFI, grip force, and pinch grip force showed large sensitivity for improvement in DAS28 and HAQ (SMR = 0.8-0.9). GAT showed modest sensitivity (SMR = 0.6-0.7). CONCLUSIONS: Patients with advanced RA attained considerable improvement in hand function that was only partly reflected by measures of general disability and disease activity. Focused assessment of hand function is therefore important for optimal evaluation of treatment response.

Daily activities and hand function in women with scleroderma.

OBJECTIVE: To give a detailed description of hand function and the ability to perform daily activities among patients with systemic sclerosis. METHODS: Thirty patients with scleroderma answered a self-administered activities of daily living (ADL) questionnaire. Hand function was estimated with respect to hand mobility, dexterity, and grip force. Perceived symptoms were reported on a visual analogue scale (VAS), and skin thickness was assessed by means of the modified Rodnan skin score. RESULTS: Finger flexion and extension were the most impaired aspects of hand mobility. Dexterity was on average reduced to 68-80%, and grip force to 46-65% compared with values for healthy persons. The predominant self-perceived problem was Raynaud's phenomenon, whereas pitting scars/ulcers were perceived as the least problem. Activities building on hand and arm function were harder to perform than activities depending on lower limb function. Raynaud's phenomenon, stiffness, grip force, and dexterity were factors with the strongest associations with ADL difficulties. Assistive devices and alternative working methods improved ADL ability. CONCLUSION: Impaired hand function was related to ADL difficulties and the use of assistive devices improved ADL. This emphasizes the importance of treatments that improve hand function and of testing ergonomic tools.

Hand Mobility in Scleroderma (HAMIS) test: the reliability of a novel hand function test.

OBJECTIVE: Hand Mobility in Scleroderma (HAMIS) is a new hand function test developed for adults who have systemic sclerosis. HAMIS consists of 9 items designed to measure all movements assessed in an ordinary range of motion (ROM)-measured hand test. The aim of this study was to examine the reliability of the test. METHODS: Two observers (one occupational therapist and one physiotherapist) performed the assessments independently of each other on 30 adult subjects. There were 25 women and 5 men in the sample (mean age 53 years, average time since diagnosis 4 years). Subjects were tested twice by each rather. RESULTS: Internal consistency ranged from 0.80 to 0.85 (Cronbach's alpha). Agreement between the two observers was good for all items (estimated kappa 0.52 to 1.00). The agreement between the observers' first and second assessments was moderate to very good for most items (estimated kappa 0.48 to 1.00), but not for the assessment of supination (estimated kappa 0.25 to 0.59). CONCLUSION: HAMIS is a reliable instrument for evaluation of hand function on scleroderma patients.

Validity of HAMIS: a test of hand mobility in scleroderma.

OBJECTIVE: Hand Mobility in Scleroderma (HAMIS) is a hand function test for persons who have systemic sclerosis (scleroderma). The purpose of HAMIS is to obtain an estimation of the hand mobility that is precise enough to detect limitation of motion at the same time as it indicates the ability to use the hand in daily occupations. The aim of this study was to test psychometric properties of the HAMIS, and the following aspects of HAMIS were examined: 1) the applicability of HAMIS, 2) concurrent validity, and 3) discriminating ability. METHODS: Forty-five patients with scleroderma were assessed for range of motion (ROM), HAMIS, and skin thickness. In addition, 15 healthy individuals completed HAMIS. RESULTS: The applicability of HAMIS was good for items assessing finger and thumb mobility and moderate for items assessing mobility of the wrist and the forearm. The relationships of HAMIS to ROM and skin score were statistically significant for all items except for pronation and supination of the forearm. There were also statistically significant differences between the patients and the healthy individuals for all items except these two. CONCLUSION: HAMIS has a demonstrated concurrent validity compared with ROM and skin score, and it showed a good ability to discriminate between healthy individuals and persons with scleroderma, although a lack of variation in the items measuring pronation and supination inferred worse psychometric properties for these two items.