Development and validation of a patient-reported outcome measure for systemic sclerosis: the EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire.

OBJECTIVES: Patient-reported outcome measures (PROMs) are important for clinical practice and research. Given the high unmet need, our aim was to develop a comprehensive PROM for systemic sclerosis (SSc), jointly with patient experts. METHODS: This European Alliance of Associations for Rheumatology (EULAR)-endorsed project involved 11 European SSc centres. Relevant health dimensions were chosen and prioritised by patients. The resulting Systemic Sclerosis Impact of Disease (ScleroID) questionnaire was subsequently weighted and validated by Outcome Measures in Rheumatology criteria in an observational cohort study, cross-sectionally and longitudinally. As comparators, SSc-Health Assessment Questionnaire (HAQ), EuroQol Five Dimensional (EQ-5D), Short Form-36 (SF-36) were included. RESULTS: Initially, 17 health dimensions were selected and prioritised. The top 10 health dimensions were selected for the ScleroID questionnaire. Importantly, Raynaud's phenomenon, impaired hand function, pain and fatigue had the highest patient-reported disease impact. The validation cohort study included 472 patients with a baseline visit, from which 109 had a test-retest reliability visit and 113 had a follow-up visit (85% female, 38% diffuse SSc, mean age 58 years, mean disease duration 9 years). The total ScleroID score showed strong Pearson correlation coefficients with comparators (SSc-HAQ, 0.73; Patient's global assessment, Visual Analogue Scale 0.77; HAQ-Disability Index, 0.62; SF-36 physical score, -0.62; each p<0.001). The internal consistency was strong: Cronbach's alpha was 0.87, similar to SSc-HAQ (0.88) and higher than EQ-5D (0.77). The ScleroID had excellent reliability and good sensitivity to change, superior to all comparators (intraclass correlation coefficient 0.84; standardised response mean 0.57). CONCLUSIONS: We have developed and validated the EULAR ScleroID, which is a novel, brief, disease-specific, patient-derived, disease impact PROM, suitable for research and clinical use in SSc.

Validity and reliability of the Patient Health Questionnaire-8 in Swedish for individuals with systemic sclerosis.

BACKGROUND: Depressive symptoms are common in rheumatic diseases and influence patients' quality of life. The Patient Health Questionnaire-9 (PHQ-9), which assesses symptoms of depression, is valid in English in patients with systemic sclerosis (SSc). However, the measurement properties of the PHQ-8 (short version of the PHQ-9) have not been evaluated in Swedish patients with SSc. OBJECTIVE: To investigate different aspects of validity and reliability of the PHQ-8 in Swedish (PHQ-8 Swe) for individuals with SSc. METHODS: A total of 101 patients with SSc participated. Content validity was evaluated via interviews of 11 patients and 10 health professionals. Construct validity, internal consistency test-retest reliability, and floor/ceiling effects were evaluated in 90 patients. RESULTS: Content validity was satisfactory, but some linguistic adjustments were made. Confirmatory factor analysis supported a better fit for a two-factor structure. Moderate-to-strong correlations were found between the PHQ-8 Swe and scleroderma HAQ including VAS (rs = 0.4-0.7); Multidimensional Assessment of Fatigue (rs = 0.7); RAND-36 subscales (rs = - 0.5 to - 0.8); and lung disease severity (Medsger scores) (rs = 0.4). There were weak correlations (rs = <0.4) between the PHQ-8 Swe and modified Rodnan skin score; and vascular, heart, and kidney disease severity. Cronbach's alpha was 0.85, corrected item-to-total correlations were >0.40, and the ICC for the total score was 0.83. No floor/ceiling effects were found. CONCLUSION: The PHQ-8 Swe has satisfactory content validity and sufficient reliability in patients with in majority limited SSc. It is more strongly associated with self-reported disability, pain, disease interferences with daily activities, fatigue, and quality of life than with disease severity, except for a moderate association with lung severity.

Common measure of quality of life for people with systemic sclerosis across seven European countries: a cross-sectional study.

OBJECTIVES: The aim of this study was to adapt the Systemic Sclerosis Quality of Life Questionnaire (SScQoL) into six European cultures and validate it as a common measure of quality of life in systemic sclerosis (SSc). METHODS: This was a seven-country (Germany, France, Italy, Poland, Spain, Sweden and UK) cross-sectional study. A forward-backward translation process was used to adapt the English SScQoL into target languages. SScQoL was completed by patients with SSc, then data were validated against the Rasch model. To correct local response dependency, items were grouped into the following subscales: function, emotion, sleep, social and pain and reanalysed for fit to the model, unidimensionality and cross-cultural equivalence. RESULTS: The adaptation of the SScQoL was seamless in all countries except Germany. Cross-cultural validation included 1080 patients with a mean age 58.0 years (SD 13.9) and 87% were women. Local dependency was evident in individual country data. Grouping items into testlets corrected the local dependency in most country specific data. Fit to the model, reliability and unidimensionality was achieved in six-country data after cross-cultural adjustment for Italy in the social subscale. The SScQoL was then calibrated into an interval level scale. CONCLUSION: The individual SScQoL items have translated well into five languages and overall, the scale maintained its construct validity, working well as a five-subscale questionnaire. Measures of quality of life in SSc can be directly compared across five countries (France, Poland Spain, Sweden and UK). Data from Italy are also comparable with the other five countries although require an adjustment.

Linguistic validation and cultural adaptation of an English version of the evaluation of daily activity questionnaire in rheumatoid arthritis.

BACKGROUND: To linguistically validate and culturally adapt the Evaluation of Daily Activity Questionnaire (EDAQ) for use in rheumatoid arthritis (RA) from Swedish to British English. The EDAQ is a patient reported outcome measure of daily activity ability. It includes 11 activity domains (Eating and Drinking; Personal Care; Dressing; Bathing; Cooking; Moving Indoors; House Cleaning; Laundry; Moving and Transfers; Communication; Moving Outdoors) and was developed for use in rheumatoid arthritis (RA). METHODS: The EDAQ was translated from Swedish to English using standard methods. Activity diaries, cognitive debriefing interviews and focus groups were completed with people with RA to: generate new culturally applicable items; identify important items in the Swedish version to retain in the English version; and develop the English EDAQ based on their views of content and layout. Content validity was established by linking the EDAQ to the International Classification of Functioning RA Core Set. RESULTS: The English EDAQ translation was harmonized with the Swedish version to ensure equivalence of meaning. Sixty-one people with RA participated. 156 activities were identified from 31 activity diaries and included in a draft English EDAQ. Following interviews (n = 20) and four focus groups, 138 activities were retained and three additional domains added (Gardening/Household Maintenance; Caring; and Leisure/Social Activities). Most ICF RA Core Set activities are in the EDAQ. CONCLUSIONS: The English EDAQ is a detailed self-report measure of ability in RA with good content validity.

Validation of the Swedish version of PROMIS-29v2 and FACIT-Dyspnea Index in patients with systemic sclerosis.

PURPOSE: To evaluate the reliability, internal consistency, and construct validity of the Swedish versions of PROMIS-29 and Functional Assessment of Chronic Illness Therapy-Dyspnea (FACIT-Dyspnea) instruments in patients with systemic sclerosis (SSc). METHODS: In a cross-sectional study, consecutive SSc patients completed a paper-based survey. Internal consistency was assessed using Cronbach's alpha. Test-retest reliability was tested employing weighted Kappa (Kw) and intra-class correlation coefficient (ICC). Construct validity was evaluated by hypotheses testing using RAND-36, MRC Dyspnea score, Scleroderma Health Assessment Questionnaire (SHAQ) and clinical measurements. RESULTS: Forty-nine patients (86% female; 73% limited cutaneous SSc) completed the survey. The mean disease duration was 11 years and mean SHAQ was 0.5. Internal consistency and test-retest reliability were good with the exception of PROMIS-29 anxiety. PROMIS-29, FACIT-Dyspnea, and Functional limitation showed strong correlations to corresponding RAND-36 domains (|rs|=0.67 to -0.85). Relevant PROMIS-29 domains, FACIT-Dyspnea and Functional limitation correlated strongly to SHAQ and VAS overall disease severity (|rs|=0.60 to -0.75). Ceiling effects (>15%) were found in six PROMIS-29 domains and in both FACIT-Dyspnea and Functional limitations. Four (4/5) hypotheses were confirmed. CONCLUSIONS: PROMIS-29 and FACIT-Dyspnea meet the requirements for reliability and have adequate construct validity in Swedish patients with SSc.Implications for rehabilitationPROMIS-29v2 and Functional Assessment of Chronic Illness Therapy-Dyspnea (FACIT-Dyspnea) Index are patient outcome measures that gain increasing interest for the evaluation of patient with rheumatologic diseases.PROMIS-29v2 and FACIT-Dyspnea Index meet the requirements for reliability and have adequate construct validity compared to legacy measures in Swedish patients with systemic sclerosis.Translation and validation of PROMs is important for studies of rare diseases in multi-center collaborations.

Managing work life with systemic sclerosis.

OBJECTIVE: To explore how individuals with SSc manage their work life. METHODS: We conducted four focus group interviews, which included 17 patients currently working at least 20 h per week. The interviews were recorded and transcribed verbatim. The transcribed texts were analysed according to thematic content analysis. Relevant statements that generated preliminary categories were identified, after which themes and underlying subthemes were generated. RESULTS: The participants perceived their work role as being important, giving them a structure in everyday life and a sense of being useful members of society. Work and private life were interacting, and different adjustments had been developed to create a satisfactory balance of activities in daily life. Three themes emerged: adjustment of work situation, adapting to own resources and disclosing limitations. Reduced working hours, flexibility in the workplace concerning time schedule, room and tasks were all valuable adjustments, and were dependent on the employer, fellow workers as well as the individual's attitude towards informing employer and colleagues about his/her limitations. The participants had simplified and rationalized a lot of household chores and pointed to the importance of prioritizing meaningful activities, as well as the necessity of having time for rest and recovery. CONCLUSIONS: Reduced working hours, work flexibility and prioritizing meaningful activities and recovery were important factors in order to manage work life. Intervention, including problem-solving techniques and re-designing of activities in daily life, could be useful to coach individuals towards finding a balance in their work life.

Individualized outcome measures of daily activities are sensitive tools for evaluating hand surgery in rheumatic diseases.

OBJECTIVES: To explore the ability of six outcome measures to capture clinically important changes in patients with rheumatic diseases undergoing hand surgery and to study predictors of changes in activity performance in different patient and surgery strata. METHODS: A total of 172 patients (median age 59 years, disease duration 18 years) were stratified into subgroups: diagnosis, age, general function, type of surgery. Performance of daily activities and satisfaction were assessed by the Canadian Occupational Performance Measure (COPM). Clinically important improvement was defined as a two-step improvement in COPM. Hand function was assessed by reference to grip strength (Grippit), pinch strength (pinch gauge), hand pain (visual analogue scale) and grip ability (Grip Ability Test). Responsiveness was calculated as effect size (ES) at 6-month follow-up compared with baseline. RESULTS: Clinically important improvement was reached by 25-69% depending on outcome measure and type of surgery. Improvement was smaller in patients with multiple simultaneous procedures. Regardless of diagnosis, age, general function and type of surgery, patients improved significantly in all measures, with the largest changes in COPM(performance) and COPM(satisfaction) (ES 0.7-1.9). The ES of pain ranged from 0.2 to 0.7, Grippit from 0.1 to 0.5 and pinch gauge from 0.4 to 0.8. Hand pain was the only significant predictor of clinically important improvement of COPM(performance): odds ratio 0.71, 95% CI 0.51, 0.98 (P = 0.041). CONCLUSION: COPM was the most sensitive instrument to capture clinically important improvement, and hand pain was a significant predictor of improvement, irrespective of diagnosis, age, general functional level and type of surgery.

Concepts of functioning and health important to people with systemic sclerosis: a qualitative study in four European countries.

OBJECTIVE: To describe the experiences of people with systemic sclerosis (SSc) in different European countries of functioning and health and to link these experiences to the WHO International Classification of Functioning, Disability and Health (ICF) to develop a common understanding from a bio-psycho-social perspective. METHOD: A qualitative multicentre study with focus-group interviews was performed in four European countries: Austria, Romania, Sweden and Switzerland. The qualitative data analysis followed a modified form of 'meaning condensation' and the concepts that emerged in the analysis were linked to the ICF. RESULTS: 63 people with SSc participated in 13 focus groups. In total, 86 concepts were identified. 32 (37%) of these were linked to the ICF component body functions and structures, 21 (24%) to activities and participation, 26 (30%) to environmental factors, 6 (7%) to personal factors and 1 (1%) to the health condition itself. 19 concepts (22%) were identified in all four countries and included impaired hand function, household activities, paid work, drugs, climate and coldness, support from others and experiences with healthcare institutions, non-pharmacological treatment, social security and benefits. CONCLUSION: Concepts identified in all four countries could be used for guiding clinical assessment, as well as interdisciplinary team care and rheumatological rehabilitation for patients with SSc. For a full understanding of the aspects of the disease that were most relevant to people with SSc, people with SSc from multiple countries needed to be involved.

Pain, fatigue and hand function closely correlated to work ability and employment status in systemic sclerosis.

OBJECTIVE: To identify factors, individual and work related, influencing work ability, and to assess the association between work ability and employment status, activities of daily life (ADLs) and quality of life in patients with SSc. METHODS: Fifty-seven consecutive patients (53 females/4 males) with SSc (47 lcSSc/10 dcSSc) were included. Median age was 58 [interquartile range (IQR) 47-62] years and disease duration 14 (9-19) years. The patients were assessed for socio-demographic characteristics, disease parameters, symptoms, work ability, empowerment and adaptations in a workplace, social support, ADLs and quality of life. RESULTS: Work ability, assessed with the Work Ability Index (WAI) could be evaluated in 48 of 57 patients. The correlation between employment status and WAI was good (r(s) = 0.79, P < 0.001). Thirteen patients had good or excellent WAI, 15 had less good and 20 had poor WAI. There were no significant differences between subgroups of WAI and socio-demographic characteristics, disease duration or degree of skin and lung involvement. However, patients with good WAI expressed milder perceived symptoms (pain, fatigue and impaired hand function; P < 0.001). Patients with better WAI had better competence (P < 0.001), better possibility of adaptations at work (P < 0.01) and impact at work (P < 0.01) than those with poorer WAI. CONCLUSIONS: In SSc, pain, fatigue and impaired hand function have a dominant impact on the WAI. Employment interventions should include support in job adaptations as well as self-management strategies to help patients deal with pain and fatigue and to enhance the confidence to perform their work.

Measures and time points relevant for post-surgical follow-up in patients with inflammatory arthritis: a pilot study.

BACKGROUND: Rheumatic diseases commonly affect joints and other structures in the hand. Surgery is a traditional way to treat hand problems in inflammatory rheumatic diseases with the purposes of pain relief, restore function and prevent progression. There are numerous measures to choose from, and a combination of outcome measures is recommended. This study evaluated if instruments commonly used in rheumatologic clinical practice are suitable to measure outcome of hand surgery and to identify time points relevant for follow-up. METHODS: Thirty-one patients (median age 56 years, median disease duration 15 years) with inflammatory rheumatic disease and need for post-surgical occupational therapy intervention formed this pilot study group. Hand function was assessed regarding grip strength (Grippit), pain (VAS), range of motion (ROM) (Signals of Functional Impairment (SOFI)) and grip ability (Grip Ability Test (GAT)). Activities of daily life (ADL) were assessed by means of Disabilities of the Arm, Shoulder and Hand Outcome (DASH) and Canadian Occupational Performance Measure (COPM). The instruments were evaluated by responsiveness and feasibility; follow-up points were 0, 3, 6 and 12 months. RESULTS: All instruments showed significant change at one or more follow-up points. Satisfaction with activities (COPM) showed the best responsiveness (SMR>0.8), while ROM measured with SOFI had low responsiveness at most follow-up time points. The responsiveness of the instruments was stable between 6 and 12 month follow-up which imply that 6 month is an appropriate time for evaluating short-term effect of hand surgery in rheumatic diseases. CONCLUSION: We suggest a core set of instruments measuring pain, grip strength, grip ability, perceived symptoms and self-defined daily activities. This study has shown that VAS pain, the Grippit instrument, GAT, DASH symptom scale and COPM are suitable outcome instruments for hand surgery, while SOFI may be a more insensitive test. However, the feasibility of this protocol in clinical practice awaits prospective studies.

Daily occupations--performance, satisfaction and time use, and relations with well-being in women with limited systemic sclerosis.

PURPOSE: To describe activity level, operationalized as ADL capacity and performance of occupations, and occupational balance, operationalized as time use and satisfaction with occupations, in women with limited systemic sclerosis, and to investigate the relationship between these occupational factors and disease characteristics, demographic variables and wellbeing. METHOD: Forty-four women of working age and with long-standing limited systemic sclerosis were assessed regarding occupational factors, clinical variables, sociodemographic characteristics and well-being. RESULTS: The women exhibited a generally high activity level and were satisfied with their daily occupations. More time spent working was related to spending less time on household chores, greater satisfaction with occupations in general and greater well-being. Symptoms such as breathlessness, fatigue and pain, influenced ADL capacity and satisfaction with occupations negatively. CONCLUSIONS: Work seems to be an important factor for satisfaction with occupations and well-being. This emphasizes the importance of investigating risk factors for work disability in people with systemic sclerosis, and of developing strategies for people with a work disability to enable them to maintain routines and a structured day. The findings also demonstrate the importance of reinforcing performance in satisfying occupations in order to maintain a feeling of well-being.

Psychometric properties of the Satisfaction with Daily Occupations (SDO) instrument and the Manchester Short Assessment of Quality of Life (MANSA) in women with scleroderma and without known illness.

This study investigated whether two instruments devised for people with mental illness, the Satisfaction with Daily Occupations (SDO) instrument and the Manchester Short Assessment of Quality of Life (MANSA), showed appropriate psychometric properties in terms of internal consistency, convergent/divergent validity, and discriminant validity when used with other samples. The study group comprised two female samples, one with physical disability (scleroderma) and one reference sample without known illness. It was hypothesized that the associations from SDO would be low or moderate to both general life satisfaction and self-rated health. The results confirmed that the associations were equal in size in both samples, but still the relationship to general life satisfaction in the scleroderma sample was somewhat higher than expected. Regarding the MANSA quality of life, the hypotheses were that the quality of life-index would show high correlations with general life satisfaction and moderate with self-rated health, and these hypotheses were confirmed for the reference sample, indicating that quality of life as measured by the MANSA converged with general life satisfaction but mainly diverged from self-rated health. In the scleroderma sample, the association to health was higher than expected. Both instruments appeared to reflect constructs that were stable across the two investigated groups, and both measures could distinguish the disability group from the healthy group. The SDO obtained a good value on internal consistency in the sample with scleroderma but a somewhat low value in the reference group, while the quality of life aspect of the MANSA exhibited good internal consistency in both samples. The instruments showed promising properties, indicating that they could be used for the target groups. However, both measures need further testing of psychometric properties.

The Modified Hand Mobility in Scleroderma Test and Skin Involvement - A Followup Study.

OBJECTIVE: To study the change in the modified Hand Mobility in Scleroderma (mHAMIS) test from early to advanced stages of systemic sclerosis (SSc), and the relationship between mHAMIS and skin involvement during followup. METHODS: This retrospective study includes 65 patients with baseline disease duration of ≤ 3 years who were assessed with the mHAMIS test at baseline and at 1 or 2 predefined followup points (3.1-5 yrs and 5.1-9 yrs after disease onset). Studied measures were the modified Rodnan skin score (mRSS), mRSS of the hand, serum cartilage oligomeric matrix protein, and digital vascular lesions. RESULTS: The mHAMIS and the mRSS hand changed synchronously during the first 5 years after disease onset (rs = 0.44, p = 0.001). In the group with high mHAMIS at baseline, both mHAMIS and mRSS hand improved significantly at the first followup (p < 0.05), and the improvement sustained during the followup in the mRSS hand. Patients with antitopoisomerase I and anti-RNA polymerase III antibodies had significantly higher mHAMIS at baseline (p = 0.003) and at the second followup (p = 0.030) compared to patients with anticentromere antibodies. Patients with digital vascular lesions at baseline had significantly higher mHAMIS during the followup (p < 0.05) compared to patients without. The mHAMIS improved significantly during the followup in patients with immunosuppressive treatment in early disease (p < 0.05), but not in patients without this treatment. CONCLUSION: The mHAMIS reflects disease activity in fibrosis in early stages of SSc. In later stages it can be regarded as a measure of damage arising from fibrotic and vascular involvement, making it suitable as an endpoint in followup examinations.

Daily occupations and well-being in women with limited cutaneous systemic sclerosis.

OBJECTIVE: This study investigated occupational performance, well-being (operationalized as general life satisfaction, domain-specific life satisfaction, and self-rated health), and perceived symptoms in women with limited scleroderma and healthy controls. METHODS: Interview-based and self-administered questionnaires were used with 36 women with limited scleroderma and 40 healthy women. RESULTS: In the scleroderma group, most of occupation problems were perceived in work and household chores. The women with scleroderma were mainly satisfied with self-care and least satisfied with household chores. Regarding domain-specific life satisfaction, the women were least satisfied with physical health and leisure. General life satisfaction showed the strongest relations to performance of self-defined occupations and satisfaction with leisure, whereas the strongest association with self-rated health was found for satisfaction with work. Fatigue was perceived as a dominant problem and was significantly associated with well-being. Furthermore, the women with scleroderma felt lower satisfaction with daily occupations and well-being than the healthy women. CONCLUSION: Loss of occupations, low satisfaction with leisure, perceived fatigue, shortness of breath, and pain indicated poorer well-being in women with scleroderma and need to be focused on in occupational therapy interventions.

High frequency ultrasound of skin involvement in systemic sclerosis - a follow-up study.

INTRODUCTION: High-frequency ultrasound offers a potential for objective and quantitative assessment of skin thickness and skin echogenicity in systemic sclerosis (SSc). Few studies have however assessed the longitudinal changes of skin involvement using ultrasound. The aim of the study was to investigate changes in skin thickness in early SSc using high frequency ultrasound during one year of follow-up in comparison to other measurements of skin fibrosis. METHODS: This retrospective study comprised 75 consecutive patients with disease duration shorter than 3 years, in whom ultrasound examination of skin thickness was performed at baseline and at the one year follow-up at five predefined sites. RESULTS: Repeated ultrasound examination identified significant changes in a majority of patients. In 21 patients, the total sum of skin thickness (TST) increased, while TST decreased in 37 patients. On a group level there were significant decreases in skin thickness of the chest (p = 0.024) and in the TST (p = 0.011) during the observation time. Both baseline and follow-up TST correlated to serum-COMP (rS: 0.41; p = 0.001; rS: 0.49; p < 0.001), modified Rodnan skin score (mRSS; rS: 0.48; p < 0.001; rS: 0.48; p < 0.001) and hand mobility in scleroderma (HAMIS; rS: 0.30; p = 0.043; rS: 0.64; p < 0.001). Changes in TST correlated with changes in serum-COMP (rS: 0.30; p = 0.034), changes in mRSS (rS: 0.43; p < 0.001) and changes in HAMIS (rS: 0.53; p = 0.001) during follow-up. CONCLUSIONS: In early SSc, skin thickness measured by high frequency ultrasound develops in parallel with serum-COMP, mRSS and the HAMIS test. Ultrasound examination of the skin allows for objective assessment of one facet of the complex process of skin fibrosis in early SSc.

Work Disability in Early Systemic Sclerosis: A Longitudinal Population-based Cohort Study.

OBJECTIVE: To study work disability (WD) with reference to levels of sick leave and disability pension in early systemic sclerosis (SSc). METHODS: Patients with SSc living in the southern part of Sweden with onset of their first non-Raynaud symptom between 2003 and 2009 and with a followup of 36 months were included in a longitudinal study. Thirty-two patients (26 women, 24 with limited SSc) with a median age of 47.5 years (interquartile range 43-53) were identified. WD was calculated in 30-day intervals from 12 months prior to disease onset until 36 months after, presented as the prevalence of WD per year (0-3) and as the period prevalence of mean net days per month (± SD). Comparisons were made between patients with different disease severity and sociodemographic characteristics, and between patients and a reference group (RG) from the general population. RESULTS: Seventy-eight percent had no WD 1 year prior to disease onset, which decreased to 47% after 3 years. The relative risk for WD in patients with SSc compared with RG was 0.95 (95% CI 0.39-2.33) at diagnosis, and increased to 2.41 (1.28-4.55) after 3 years. There were no significant correlations between WD and disease severity, but between WD and years at workplace (rs = -0.72; p = 0.002), education (rs = -0.51; p = 0.004), and sickness absence the month before disease onset (rs = 0.58; p = 0.001), respectively. CONCLUSION: Considerable increase in WD was noted 3 years after disease onset. Limited education, fewer years at workplace, and sickness absence before disease onset may be risk factors for sustained WD.

Evaluation of paraffin bath treatment in patients with systemic sclerosis.

OBJECTIVE: To investigate the effects of treatment with paraffin bath in patients with systemic sclerosis (scleroderma). METHODS: In 17 patients with scleroderma one hand was treated daily with paraffin bath in combination with hand exercise. The other hand was treated with exercise only and was considered a control. Hand function was estimated before treatment and after 1 month of treatment, concerning hand mobility and grip force, and perceived pain, stiffness and skin elasticity. RESULTS: At the follow-up, finger flexion and extension, thumb abduction, volar flexion in the wrist, and perceived stiffness and skin elasticity had improved significantly in the paraffin-treated hand compared with the baseline values. The improved hand function was independent of skin score and disease duration. Improvements in function were significantly greater in the hand which was treated with paraffin bath and exercise than in the hand treated with exercise only concerning extension deficit, perceived stiffness and skin elasticity. CONCLUSIONS: In this pilot study hand exercise in combination with paraffin bath seemed to improve mobility, perceived stiffness and skin elasticity. However, further studies with larger sample size are needed to attain more reliable results of the effect of paraffin bath treatment in patients with scleroderma.

Development of a modified hand mobility in scleroderma (HAMIS) test and its potential as an outcome measure in systemic sclerosis.

OBJECTIVE: To modify the hand mobility in scleroderma (HAMIS) test by reducing the number of items and amount of equipment needed, and to evaluate the construct validity of this modified HAMIS (mHAMIS). METHODS: Our retrospective study is based on 266 patients previously examined using the original HAMIS test. Data were divided into 3 groups depending on disease duration after onset: (1) 0-3 years, (2) 3.1-5 years, and (3) 5.1-9 years. Disease variables included were skin involvement using the disease subset and the modified Rodnan skin score (mRSS), and digital lesions. Cronbach's alpha coefficient was calculated separately for limited (lcSSc) and diffuse systemic sclerosis (dcSSc) for the right and left hand, and for the groups with different disease duration. The construct validity of the mHAMIS was assessed by searching for a correlation with hand skin score. RESULTS: An mHAMIS test consisting of finger flexion, finger extension, finger abduction, and dorsal extension was developed. The internal consistency of this test was 0.78, 0.83, and 0.73 in the 3 groups with different disease duration. In the whole study group, mHAMIS showed a significant correlation with mRSS and hand skin score (rs=0.39 and 0.43, respectively), and was able to discriminate between lcSSc and dcSSc (p=0.001), and between patients with and without ulcers (p=0.015). CONCLUSION: The mHAMIS involves 4 easily measurable items and has the potential to be a relevant clinical measure of outcome in the evaluation of fibrotic skin involvement in SSc.

Hand function and performance of daily activities in systemic lupus erythematosus.

OBJECTIVE: To investigate hand problems in patients with systemic lupus erythematosus (SLE) and to explore consequences on the ability to perform daily activities. METHODS: One hundred nine patients with SLE completed a questionnaire assessing hand problems in terms of deficits in body structures, e.g., joints, and body functions including pain, grip force, and other physiologic functions of the hand, the Health Assessment Questionnaire (HAQ), and the Simple hand test. Patients who stated problems in hand function answered questions about performance of daily activities and to what extent different deficits in body structures and body functions interfered. RESULTS: Seventy-three percent of patients experienced hand problems and 42% reported interference with performance of daily activities. Problems from body structures of the hand were distributed relatively evenly over joints and tendons/muscles. Reduced grip force and activity-induced pain were the most commonly reported problems in body functions. The most affected activity area was productivity, namely household tasks, work at home, work/study, and child care; least affected was self-care. Reduced grip force followed by fumbling and pain were the most frequently reported body functions to create difficulties in performing daily activities. When comparing patients with and without difficulties in performing daily activities, there were significant differences in problems from tendons/muscles, joints in the thumb, reduced force, stiffness, fumbling, numbness/tingling, and the HAQ. CONCLUSION: A majority of the study group had hand problems and almost half of the group experienced difficulties in performing daily activities due to SLE. The most affected activity area was productivity, where reduced grip force, fumbling, and pain were the most interfering body functions.