Systemic Sclerosis Questionnaire Research Articles

Impact of foot functionality in patients with systemic sclerosis: Cross-sectional study

BackgroundProgressive systemic sclerosis or systemic scleroderma (SS) is a chronic and rare autoimmune disease that mainly affects the skin and various internal organs. Raynaud's phenomenon and digital ulcers are some of the symptoms that affect the foot, causing a decrease in the quality of life of patients. The objective of this study is to determine the functionality of the feet in patients with SS and determine the impact on their daily lives. MethodsA sample of 165 patients (154 women, 11 men) diagnosed with SS with a mean age of 46.29 ± 11.36 years and a mean body mass index (BMI) of 24.90 ± 5.77 was recruited. Each participant completed the Foot Function Index (FFI) questionnaire and the Systemic Sclerosis Questionnaire (SySQ). A multivariate analysis was performed to determine which factors were related to a higher score in both questionnaires. Results32.1% of the participants (n = 53) had claw toe deformities, 79.4% (n = 131) Raynaud's disease and 20% (n = 33) a history of foot ulcers. 51.5% of the participants (n = 85) presented symptoms in their nails, the most frequent sign being thickening, hardening and yellow coloration. The final score of the FFI questionnaire was 3.51 ± 2.41 (0–9.9), the pain subscale being the highest, with a score of 5.06 ± 2.75, followed by foot disability (3.26 ± 2.91) and difficulty performing activities (1.55 ± 2.22). The final score of the SySQ questionnaire was 0.95 ± 0.45 (0.18–2.45), and the subscales with the highest score were symptom frequency (1.30 ± 0.47), symptom intensity (1.11 ± 0.55), and general skill limitation (0.47 ± 0.51). A high correlation was observed between the final FFI score and the final SySQ score (r = 0.712; p=<0.001). Also, between foot activity limitation and general skill limitation (r = 0.658; p=<0.001). A moderate correlation was observed between foot pain score and overall symptom intensity (r = 0.482; p=<0.001). Also, between foot disability and overall symptom frequency (r = 0.556; p=<0.001). The multivariate analysis (R2 0.51) showed that the final FFI score had a significant relationship with the final SySQ score (p < 0.001). No significant correlation was found between age (p = 0.15), gender (p = 0.49), BMI (p = 0.74) or time of diagnosis (p = 0.57) and FFI. ConclusionSS is a disease that affects foot functionality in patients, with a greater impact on the pain scale. There is a correlation between the final FFI score and the final SySQ score, so improving foot functionality could help to improve the overall functionality of the patient with sclerosis.

The effect of a self-management program on the quality of life of patients with scleroderma.

Scleroderma is a rare disease with complex disorders. It affects the quality of life with severe impacts on the skin and extensive complications in the internal organs, and does not have a definitive treatment. This study aimed to investigate the effect of a self-management program on the quality of life of patients with scleroderma. This was a clinical trial in which 54 patients with scleroderma were randomly divided into two groups of 27 each (experimental and control groups). The data were collected using the Systemic Sclerosis Questionnaire. A self-management program was sent to the experimental group via a mobile phone application (WhatsApp) every day for three months. Statistical analysis was performed in Statistical Package for the Social Sciences V21. The Wilcoxon signed-rank test showed that the average overall quality of life score of the experimental group showed a significant increase after the implementation of the program (P value: 0.00). The average overall quality of life score of the control group also significantly declined after the intervention (P value: 0.00). The Mann-Whitney U test revealed that there was no significant difference in the overall quality of life score of the two groups before the intervention (P value: 0.31); however, after the implementation of the self-management program, a significant difference was observed between the two groups (P value: 0.00). According to the results, the self-management program can help improve the quality of life of patients with scleroderma.

Validity and reliability of the Mouth Handicap of Systemic Sclerosis questionnaire in a UK population.

The objective of this study was to explore the psychometric properties of the Mouth Handicap in Systemic Sclerosis (MHISS) specifically exploring aspects of validity and reliability of MHISS in UK SSc patients. A total of 150 questionnaires were distributed in this study at the Oral Medicine Department of UCLHT Eastman Dental Hospital, the Outpatient Rheumatology Clinic of the Royal Free Hospital and Scleroderma Family Day. Participants were asked to complete three questionnaires (SF-36, OHIP-14 and MHISS) along with a pro forma of demographic and disease-specific questions. Both convergent validity and internal consistency reliability were examined. The convergent validity was assessed by Spearman's correlation coefficient, and internal consistency reliability was assessed by Cronbach's alpha. Of the 150 questionnaires distributed to participants, 68 respondents were included. With regard to construct validity, a low to moderate degree of convergent validity was found between MHISS total score and all SF-36 subscales. However, the total MHISS score was highly correlated to all OHIP subscales. MHISS questionnaire is a self-administered patient-reported outcome measure that assesses the mouth disability in SSc patients. However, improving the global and oral health of SSc patients is important as there is no cure for the disease; thus, instruments that record the impact of the condition and indicate the effect of treatment on the lives of patients are of paramount importance. Current results demonstrate good preliminary psychometric properties (validity and reliability) in a UK population with further exploration of psychometric properties with an emphasis on interpretability required.

Translation, validation and cross-cultural adaptation of the mouth handicap in systemic sclerosis questionnaire into the Turkish language.

The aim of this study was to translate and adapt the Mouth Handicap in Systemic Sclerosis (MHISS) Questionnaire into the Turkish language and evaluate its validity and reliability in Turkish systemic sclerosis (SSc) patients. The MHISS was translated according to Beaton guidelines. Patients being diagnosed with SSc, being between 18-65years old and receiving no treatment between test-retest assessments were included to study. Test-retest reliability was evaluated, comparing the results of two administrations, with Spearman's correlation. Internal consistency was assessed by Cronbach's α. Validity of the questionnaire was assessed by comparison with mouth opening, total scores of Disabilities of Arm, Shoulder and Hand Questionnaire (DASH) and Health Assessment Questionnaire (HAQ). Construct validity was tested by factor analysis. Forty-five SSc patients were included in the study. The Turkish version of the MHISS (MHISS-T) met set criteria of reliability and validity. Internal consistency (Cronbach's α=0.863) and test-retest reliability were excellent (r=.88). The correlations between MHISS-T and inter-incisor distance, MHISS-T and HAQ and MHISS-T and DASH were negatively and statistically significant (r=-0.739, P<.001), very good and statistically significant (r=.664, P<.001), good and statistically significant (r=.570, P<.001), respectively. Regarding factor analysis, MHISS-T has three subscales. Our results demonstrated that the Turkish version of the MHISS-T has excellent test-retest reliability and very good validity. As a result of this study we determined that MHISS-T is a valid and reliable instrument to measure mouth disabilities in Turkish-speaking SSc patients.

Mouth Handicap in Systemic Sclerosis Questionnaire (MHISS) in Turkish

Mouth Handicap in Systemic Sclerosis Questionnaire (MHISS) in Turkish

The Systemic Sclerosis Questionnaire (SySQ): Validation of the Translation of the Original German Version Into Spanish and Its Relationship to the Disease and to Quality of Life

ObjectiveTranslation, transculturation and validity of the self-administered questionnaire for functionality (Systemic Sclerosis Questionnaires [SySQ]) for use in Spanish patients with systemic sclerosis and its relationship to the severity of the disease and to quality of life. Patients and methodsWe conducted an observational analytical study to perform a cross-cultural validation of the self-administered questionnaire on functionality in scleroderma. The validity of the form and content was evaluated by an expert panel. The method included: (a) adaptation into Spanish of the construct for translation and back translation, and transculturation; (b) internal consistency with the SySQ (Cronbach's alpha), and (c) reproducibility was assessed taking into account all occasions in which the test was performed with Cohen's kappa. Additionally, we calculated the Spearman correlation coefficient with the Medsger severity scale, Health Assessment Questionnaire score and SF-36 score. ResultsWe included 70 patients with systemic sclerosis: age 17–78 (51±12) years, 65 (93%) were women, diffuse/limited subtype 64/36%, disease duration of .5–40 years. Optimal internal consistency for all categories of the final version of SySQ (Cronbach's α of .961) and intraobserver reliability in 2 tests over a 2-week interval (Cohen's kappa coefficient .618) and optimal interobserver reliability in 2 tests on the same day (Cohen's kappa coefficient .911). Moderate correlation between functionality by SySQ and by Health Assessment Questionnaire (r=.573, P<.0001). Inverse correlation between SySQ and quality of life mental health domain SF-36 (r=−.435, P<.001) and physical domain SF-36 (r=−.638, P<.001). Medsger severity scale (tendon, heart, lung, vascular) also showed significant correlation with SySQ. ConclusionsSySQ in this validated Spanish version is a suitable instrument to measure functional status in patients with systemic sclerosis. Reduced functionality is related to greater tendon and peripheral vascular involvement and to a poorer quality of life.

AB0628 Clinical Efficacy of A LIP Balm in the Aesthetic Improvement in Women Affected by Systemic Sclerosis

BackgroundSystemic sclerosis is a connective tissue disease which affects blood vessels, the skin and verious internal organs, resulting in articular deformities and possible functional compromission. The mouth, the lips and...

Translation, cultural adaptation and validation into portuguese (Brazil) in Systemic Sclerosis Questionnaire (SySQ)

IntroductionSystemic sclerosis (SSc) is a multisystem disease, autoimmune disorder char- acterized by a fibroblastic disfunction, with significant impact on quality of life (QoL), mea- sured by instruments or questionnaires that usually were formulated in other languages and in different cultural contexts. ObjectiveTranslate into Brazilian Portuguese, cross cultural adaptation and assess the reli- ability and validity of the Systemic Sclerosis Questionnaire (SySQ). MethodologyTranslation and adaptation: into Portuguese and cross-cultural adaptation was performed in accordance with studies on questionnaire translation methodology into other languages. Reliability: it was analyzed using three interviews with different inter- viewers, two on the same day (interobserver) and the third within 14 days of the first as- sessment (intraobserver).Validity was assessed by correlating clinical and quality of life parameters with the domain scores of Sysc. Statistical analysis: a descriptive analysis of the study sample. Reproducibility was assessed using an intraclass correlation coefficient (ICC). Internal consistency was assessed using Cronbach's alpha coefficient. To assess va- lidity we used Spearman correlation coefficient. Five percent was the level of significance adopted for all statistical tests. ResultsIn the evaluation of the questionnaires, the results were similar to the original questionnaire, the internal consistency ranging between 0.73 and 0.93 for each item. The interobserver reproducibility was very good for all domains (α = 0.786 to 0.983) and intrao- bserver agreement was considered very good for general symptoms domain (ICC = 0.916), good for musculoskeletal symptoms domain (ICC = 0.897) and cardiopulmonary domain (ICC = 0.842) and reasonable for gastrointestinal symptoms domain (ICC = 0.686). ConclusionThe Brazilian Portuguese version of SySQ proved to be reproducible and valid for our population, using a recognized methodology for translation and cultural adaptation of questionnaires, as well as to assess the reproducibility and validity.

Tradução, adaptação cultural e validação para a língua portuguesa (Brasil) do Systemic Sclerosis Questionnaire (SySQ)

IntroductionSystemic sclerosis (SSc) is a multisystem disease, autoimmune disorder characterized by a fibroblastic disfunction, with significant impact on quality of life (QoL), measured by instruments or questionnaires that usually were formulated in other languages and in different cultural contexts. ObjectiveTranslate into Brazilian Portuguese, cross cultural adaptation and assess the reliability and validity of the Systemic Sclerosis Questionnaire (SySQ). MethodologyTranslation and adaptation: into Portuguese and cross-cultural adaptation was performed in accordance with studies on questionnaire translation methodology into other languages. Reliability: it was analyzed using three interviews with different interviewers, two on the same day (interobserver) and the third within 14 days of the first assessment (intraobserver).Validity was assessed by correlating clinical and quality of life parameters with the domain scores of Sysc. Statistical analysis: a descriptive analysis of the study sample. Reproducibility was assessed using an intraclass correlation coefficient (ICC). Internal consistency was assessed using Cronbach's alpha coefficient. To assess validity we used Spearman correlation coefficient. Five percent was the level of significance adopted for all statistical tests. ResultsIn the evaluation of the questionnaires, the results were similar to the original questionnaire, the internal consistency ranging between 0.73 and 0.93 for each item. The interobserver reproducibility was very good for all domains (α = 0.786 to 0.983) and intraobserver agreement was considered very good for general symptoms domain (ICC = 0.916), good for musculoskeletal symptoms domain (ICC = 0.897) and cardiopulmonary domain (ICC = 0.842) and reasonable for gastrointestinal symptoms domain (ICC = 0.686). ConclusionThe Brazilian Portuguese version of SySQ proved to be reproducible and valid for our population, using a recognized methodology for translation and cultural adaptation of questionnaires, as well as to assess the reproducibility and validity.

Translation, cross-cultural adaptation, and validation of the Mouth Handicap in Systemic Sclerosis questionnaire (MHISS) into the Dutch language

The Mouth Handicap in Systemic Sclerosis (MHISS) is a French-generic questionnaire evaluating mouth-opening restriction, dryness, and esthetic concerns. The aim of this study was to translate and adapt the MHISS questionnaire into the Dutch language and evaluate its psychometric properties. The MHISS was translated according to international guidelines, field-tested among 16 systemic sclerosis (SSc) patients, and adapted. Subsequently, the Dutch MHISS was administered to 52 SSc patients visiting the outpatient or day patient clinic of a university hospital and readministered after 2 weeks. Internal consistency was tested by computing Cronbach's alpha. Test-retest reliability was determined by computing the intraclass correlation coefficient (ICC) and validity by determining associations with measures of overall functioning (Health Assessment Questionnaire (HAQ)), maximum mouth opening (MMO, in millimeter), subjective xerostomia (visual analog scale), and objective xerostomia (Saxon test). Patients had mean ± standard deviation (SD) age and disease duration of 55 ± 21 and 7.2 ± 7.3 years. Twenty-seven (52 %) patients had diffuse cutaneous SSc. The mean Dutch MHISS score was 17.5 (SD 10.0) with Cronbach's alpha being 0.862. Dutch MHISS scores differed significantly between patients with high and low disability levels (HAQ, MMO, and subjective and objective xerostomia divided according to the median; paired t test). Spearman rank correlations with HAQ (r = 0.599, p = 0.000), MMO (r = -0.518, p = 0.000), and subjective xerostomia (r = 0.536, p = 0.000) were moderate; correlation with objective xerostomia did not reach statistical significance. The ICC was 0.94. The Dutch version of the MHISS demonstrated good psychometric properties and is useful in assessing mouth disability in SSc patients.

Patientenzentriertes Assessment der funktionalen Gesundheit bei systemischer Sklerodermie?Wo stehen wir?

Self-administered patient-centered questionnaires have been shown to be practical, reliable and valid in terms of evaluating functional limitations in rheumatic diseases. In systemic sclerosis a modified version of the HAQ and condition-specific questionnaires have been used. The Health Assessment Questionnaire (HAQ) does not comprehensively cover functional limitations in patients with systemic sclerosis. Visual Analogue Scales added to the modified HAQ reflect general and organ-specific symptoms only to some extent. The Self-administered Systemic Sclerosis Questionnaire (SySQ) includes general, organ-specific and musculoskeletal symptoms with a focus on functional limitations of the upper and lower extremities. The SySQ has not been examined longitudinally nor has it been validated cross-culturally. WHO's International Classification of Functioning, Disability and Health (ICF) could serve as a future reference framework and common language in terms of the design of new disease-specific, patient-centered, comprehensive questionnaires for systemic sclerosis as well as in the further improvement of established questionnaires.

Development and validation of a self-administered systemic sclerosis questionnaire (SySQ).

To develop a self-administered systemic sclerosis questionnaire (SySQ) covering condition-specific functional limitation and symptoms. METHODS An initial item pool was generated by open patient interviews. A preliminary questionnaire was devised using 62 systemic sclerosis (SSc; scleroderma) patients. Factor analysis was used for further selection and grouping of items into distinct scales. The retrieved scales were tested for internal consistency and test-retest reliability. Spearman's rank correlation and Wilcoxon's rank sum test were used to examine hypothesized associations of the SySQ with various clinical and laboratory features. Altogether 32 SySQ items were selected and aggregated into 12 scales addressing 'pain', 'stiffness', 'coldness', 'complex functions', 'strength of hands', 'rising', 'walking', shortness of breath', 'upper airway symptoms', 'eating', 'swallowing' and 'heartburn/regurgitation'. Internal consistency ranged from 0.93 ('complex functions') to 0.73 ('heartburn/regurgitation'); Spearman's correlation coefficient for test retest reliability ranged from 0.93 to 0.73 (P < 0.001). While the scales were associated with corresponding functional impairments, there was generally less association with morphological impairments. The SySQ is a valid and reliable condition-specific measure in patients with SSc. Individually applicable scales cover a wide spectrum of general and organ-specific SSc symptoms and functional limitation. After further validation with respect to its ability to measure change, it may be used in clinical, health services and epidemiological research.