Effects of sense and functionality changes in the hands on activity and participation in patients with juvenile scleroderma.

OBJECTIVES: The purpose of the study was to examine the effects of sense and functionality changes in the hands on activity and participation in patients with juvenile scleroderma (JS). METHODS: Sixteen patients with juvenile localized scleroderma (JLS), 14 patients with Juvenile Systemic Sclerosis (JSS), and 30 healthy controls were included. Light touch-deep pressure sensation was assessed by Semmes-Weinstein monofilament test (SWMT). Localization sensation testing was performed by lightly stroking the patient's skin. The hand joint range of motion was measured with a goniometer, hand grip strength with Dynomometer, the pinch gripping force with pinch meter, and the hand mobility with modified Hand Mobility in Scleroderma (mHAMIS). Children completed their activity and participant performance status with 'Childhood Health Assessment Questionnaire (CHAQ)' and 'Jebson Taylor Hand Function Test (JTHFT)' questionnaire tests. The quality of life was evaluated using the 'Scleroderma Health Assessment Questionnaire (SHAQ)'. RESULTS: There were significantly differences among evaluated three groups in light of touch-deep pressure sensation, sense of touch localization, range of motion, mHAMIS scores, JTHFT scores, all CHAQ scores, and almost all SHAQ score (p < .05). Over than half of patients with JSS (57.1%) and almost half of patients with JLS stated that their diseases obstructed them from doing any activity (p < .001). A significant percent of JSS patients (64.3%) had hand and wrist joint involvement. CONCLUSION: Sensory and functional disorders caused by hand involvement in JS patients result in limitation of daily living activities and affect negatively the effective usage of the hand. Approximately half of the JS patients had disabilities in performing pinch motor skills of hands. The assessment of sensory symptoms that affect the functionality, activity level and participation of JSS and JLS patients should be considered during the routine clinical examination. We suggest the sensory therapies as an important factor in increasing the effectiveness of rehabilitation.

Sustained efficacy of a concise self-management programme for hands in systemic sclerosis: a longitudinal case-control observational study.

OBJECTIVES: In a longitudinal case-control observational study, we evaluated the benefits of a self-management programme for hands developed for patients with SSc. METHODS: Patients with SSc included in the intervention group (IG) received a concise self-management programme with emphasis on hand exercises and were evaluated during 24 weeks regarding hand pain, hand function, range of motion, grip and tip and key pinch strength. Results were compared with a control group (CG) with no intervention using an analysis of variance for repeated measures with variables transformed into ranks (P ≤ 0.05). Effect sizes were calculated using Cohen's test. RESULTS: Of 90 patients who were evaluated, seven were excluded at enrolment and 26 were excluded during the follow-up. Data from 57 subjects (IG 40, CG 17) were used for analysis. Groups were similar at baseline, except for the Scleroderma HAQ and tip and key pinch strength. Outcome improvements were noted only in the IG (P ≤ 0.05, large effect size). In the CG, variables did not change or had even worsened (hand grip strength and finger motion). CONCLUSIONS: This self-management programme based on hand exercises for SSc resulted in pain reduction and hand function, strength and range of motion improvement. It can be a simple and useful intervention, especially when a regular rehabilitation programme is not available.

Effect of a tailored home-based exercise program in patients with systemic sclerosis: A randomized controlled trial.

OBJECTIVE: The aim was to evaluate the effect of a home-based exercise program on functional capacity, health-related quality of life (HRQoL), and disability, in patients with systemic sclerosis (SSc). METHODS: A 6-month randomized controlled trial was conducted on SSc patients by comparing a home-based minimally supervised exercise program (exercise on a stationary cycle and strengthening of upper limbs; stretching of the hands) with usual care. At baseline and after 3 and 6 months, the patients underwent: 6 minutes walking test; hand mobility in scleroderma test; maximal exercise test on an ergocycle; strength measures (handgrip, quadriceps, and biceps). HRQoL (short-form 36 [SF-36]) and disability (health assessment questionnaire disability index [HAQ-DI]) were measured at the same time. RESULTS: Forty-four patients participated in the study. Twenty-two were randomly assigned to the intervention group (IG, mean age 63.60 ± 10.40 years) and 22 to the control group (CG, 61.80 ± 14.40 years). At 6 months, the distance walked in 6 minutes increased by 46 m (baseline 486, 95% CI 458-513 m; 6 months 532, 95% CI 504-561 m) in IG, whereas it decreased by 5 m (baseline 464, 95% CI 431-497 m; 6 months 459, 95% CI 427-490 m) in CG with a significantly different temporal trend at the between-groups comparison (P < .001). An improvement was also observed for strength measures (handgrip, P = .003; quadriceps, P < .001; biceps, P < .001), for the SF-36 physical component score (P < .001) and for the HAQ-DI (P = .011). CONCLUSIONS: This study indicates that in SSc patients, a minimally supervised home-based exercise program improves physical performance, quality of life, and disability in comparison with usual care.

Availability of EuroQol-5-Dimensions-5-Level (EQ-5D-5L) as health-related QOL assessment for Japanese systemic sclerosis patients.

Objective: To assess the functional disability in Japanese patients with systemic sclerosis (SSc) using the EuroQol-5-Domain-5-Level health questionnaire (EQ-5D-5L), which was developed in Europe to demonstrate the cost utility of treatments for non-specific diseases.Methods: The EQ-5D-5L and Disability Index of the Health Assessment Questionnaire (HAQ-DI), which is a questionnaire for the quality of life for rheumatic diseases, were completed by 109 Japanese patients with SSc, and the clinical findings and laboratory data were collected at the same time.Results: There was a correlation between the EQ-5D-5L score and HAQ-DI score. The EQ-5D-5L index score was affected by the % of predicted vital capacity (%VC), pulmonary arterial hypertension, and renal crisis. The %VC and renal crisis were also indicated as factors reducing the quality of life in the HAQ-DI. There was no difference in the EQ-5D-5L score between the SSc subtypes or among autoantibodies.Conclusion: Our single-center study demonstrated the EQ-5D-5L to be a valuable assessment tool for functional disability in Japanese SSc patients, similarly to the disease specific HAQ-DI.

Construction of an ICF core set and ICF-based questionnaire assessing activities and participation in patients with systemic sclerosis.

OBJECTIVES: To develop an International Classification of Functioning, Disability and Health (ICF) core set for SSc and to conceive a patient-centred ICF-based questionnaire assessing activities and participation in patients with SSc. METHODS: The construction of the ICF core set followed two steps. In the first step, meaningful concepts related to SSc were collected using data source triangulation from patients (n = 18), experts (n = 10) and literature (n = 174 articles). In the second step, concepts were linked to the best-matching ICF categories by one reviewer according to prespecified linking rules. Finally, patient-reported activities and participation categories of the ICF core set were translated into understandable questions. RESULTS: After linking concepts to ICF categories, 150 ICF categories were collected from focus groups, 22 from experts and 82 from literature. After fusion of the sources and removal of duplicates, the ICF core set included 164 categories: one at the first level, 157 at the second level and six at the third level, with 50 categories on body functions, 15 on body structures, 52 on activities and participation, and 47 on environmental factors. Patient-reported ICF categories on activities and participation were translated into a patient-centred ICF-based 65-item questionnaire. CONCLUSION: The present study proposes an ICF core set that offers a conceptual framework for SSc patients' care and health policy. Using a patient-centred approach, a patient-centred ICF-based questionnaire, the Cochin Scleroderma ICF-65 questionnaire, assessing activities and participation in patients with SSc, was conceived. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov, NCT01848418.

Long-term follow-up of finger passive range of motion in Japanese systemic sclerosis patients treated with self-administered stretching.

OBJECTIVE: Severe skin sclerosis in patients with systemic sclerosis (SSc) can result in a loss of hand function. The aim of this study is to examine the long-term changes of finger passive range of motion (ROM) in Japanese SSc patients treated with self-administered stretching. METHODS: This is a single-center, retrospective, observational cohort study. Forty-three Japanese patients with SSc were given instructions on self-administered stretching. ROM was assessed using a goniometer on their first visit and after 1 year, 3 years, 5 years and 9 years. Hand function was assessed by the Health Assessment Questionnaire disability index (HAQ-DI) at their first visit and after 9 years. RESULTS: Total passive ROM significantly improved in each finger after 3 years of finger stretching. Most patients (37 of 43 patients, 86%) improved or maintained total passive ROM and hand function within 9 years after their first visit. However, significant improvement of total passive ROM was lost in 6 of 43 SSc patients (14%) 9 years after their first visit. The HAQ-DI also was increased in these six patients. Multivariable analyses revealed that re-elevation of modified Rodnan total skin thickness score during the clinical course (OR = 5.260e + 7, 95% CI 1.52e + 150-uncalculated p = .0096) was the independent factor associated with deterioration of total passive ROM at 9 years. CONCLUSION: Patients with progressive skin sclerosis during the clinical course need multimodality therapy to maintain finger joint motion, since the effect of self-administered stretching is limited in these patients.

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.

Rehabilitation in systemic sclerosis: proposed personalised rehabilitation programme.

OBJECTIVES: Systemic sclerosis is a multisystem autoimmune disease that causes chronic widespread obliterative vasculopathy of the small arteries associated with various degrees of tissue fibrosis. The prognosis of the disease depends largely on the visceral involvement; however musculoskeletal involvement is an important factor to functional disability. Suffering from a chronic auto-immune disease, such as Systemic Sclerosis, compromises the quality of life and the work ability. The rehabilitative treatment may be a viable option for improving the quality of life, but there are few studies to support this hypothesis in adequate population. (Rehabilitation is still an under- studied field of research). Aim: Proposed personalised rehabilitation programme, with a multilocalized, multidisciplinary approach specifically designed for patients with Systemic Sclerosis. METHODS: Retrospective study. Setting: Rehabilitation Institute, inpatients. Population: Forty-three patients affected by systemic sclerosis. Methods: Patients underwent a rehabilitation programme of 3 weeks. Three cycles of rehabilitation in 3 consecutive years were administered. The priority outcome measure was the Health Assessment Questionnaire-Disability Index (HAQ-DI). The effectiveness of treatment cycles repeated at 1-year intervals was also compared to the first cycle. RESULTS: Each treatment had an acute beneficial effect, leading to an improvement in the disability score after each of the three cycles (admission vs discharge: 1.2±0.6 vs 0.8±0.6, p minor than 0.0001, 1.3±0.6 vs 1.0±0.6, p minor than 0.0001 and 1.4±0.7 vs 1.1±0.7, p minor than 0.0001). The improvement in HAQ-DI scores after repeated cycles was progressively lower, but the difference in efficacy was not statistically significant (p=0.38 and p=0.17 for the comparison between the second and the first and the third and the first cycles respectively). CONCLUSIONS: The multilocalized, multidisciplinary protocol developed by our rehabilitation team led to a significant reduction in disability perceived by patients, independently of the disease duration. The beneficial effects at the end of the first cycle were reproduced in the subsequent cycles. Nevertheless, there was a tendency of progressive efficacy reduction in following cycles, due to increased disability caused by disease stage. Impact of rehabilitation: We believe that our results demonstrate the utility of personalised, multilocalized, multidisciplinary rehabilitation treatment in slowing the evolution of systemic sclerosis.

Functional disability and other health-related quality-of-life domains: points to consider for clinical trials in systemic sclerosis.

Patients with SSc have the highest mortality among the rheumatic diseases. In addition, SSc is associated with disfigurement, hand contractures, fatigue, poor sleep, severe RP with numbness and tingling of the fingers can lead to decrements in quality of life. This Points to Consider article provides practical considerations for design of trials for functional disability and other health-related quality-of-life issues.

Efficacy of intensive hand physical therapy in patients with systemic sclerosis.

OBJECTIVES: To evaluate the efficacy of a three-week period of complex and intensive hand physical therapy on hand function in patients with systemic sclerosis (SSc). METHODS: Thirty-one patients with SSc were treated. Hand stretching exercises, ergotherapy supplemented with thermal and mud baths, whirlpool therapy and soft tissue massage were daily used during a three-week period. The control SSc group (n=22) received similar management for their large joints leaving out their hands. The primary outcomes of this study were the Health Assessment Questionnaire (HAQ) and the Disabilities of the Arm, Shoulder and Hand (DASH). Hand Anatomic Index (HAI), Cochin Hand Function (CHFT) and clinical characteristics were also assessed before starting the therapy and six months afterwards. RESULTS: Six months after the investigation period, only the group receiving hand physical therapy showed improvement in HAQ and DASH scores compared to the baseline values (p<0.05). The improvement in median HAQ value (25%-75% quartiles) reached the clinical meaningful rate (baseline 1.125/0.625-1.625/ versus 0.75/0.25-1.5/ at six months). Visual analogue scales of global pain (p<0.01) and Raynaud's phenomenon (p<0.05) also had better results than at baseline. HAI, gripping strength and CHFT also showed some improvement, but did not reach the significance level of change by the end of the six-month observation period. CONCLUSIONS: The complex physical therapy caused favourable changes in both the HAQ and the DASH indicating that this particular program had some long-term beneficial effect on hand function in patients with SSc.

Validity and responsiveness of the Michigan Hand Questionnaire in patients with systemic sclerosis.

OBJECTIVE: The aim was to assess the validity and responsiveness of the Michigan Hand Questionnaire (MHQ) in patients with SSc. METHODS: Data were gathered in connection with a randomized, controlled trial comparing the effectiveness of a 12-week multidisciplinary team care programme, including a hand function treatment module, with regular care. Hand function was evaluated by the MHQ (37 items, six domains: Function, Daily activities, Pain, Work, Aesthetics and Satisfaction) and other measurements, including the HAQ, Hand Mobility in Scleroderma, Sequential Occupational Dexterity Assessment (SODA), grip strength, pinch grip and modified Rodnan Skin Score. Fifty-three patients (28 intervention group and 25 control group) completed evaluation at baseline and after 12 weeks. Validity was determined by computing Spearman correlation coefficients between the baseline MHQ total score and subscales and other measures of (hand) disability. Responsiveness in the intervention group was evaluated by the standardized response mean and effect size (ES). In addition, the pooled ES for the difference between the two groups was computed. RESULTS: Significant correlations were seen between the MHQ total score and the HAQ (r = -0.62), Hand Mobility in Scleroderma (r = -0.54), SODA (r = 0.47), SODA Pain (r = 0.32) and modified Rodnan Skin Score (r = 0.46). The ES of the MHQ total score within the intervention group was 0.49, which was larger than that of all other outcome measures. Similar results were obtained for the standardized response mean. The pooled ES of the difference between intervention and control groups for the MHQ total score was 0.86. CONCLUSION: The MHQ demonstrated adequate validity and responsiveness in patients with SSc.

The relationship between skin symptoms and the scleroderma modification of the health assessment questionnaire, the modified Rodnan skin score, and skin pathology in patients with systemic sclerosis.

OBJECTIVE: To determine how well skin symptoms considered specific to SSc are captured by patient reported outcomes currently used for assessing patients with SSc, the SHAQ, or skin disease, the Skindex-29; and how well these symptoms correlate with the extent of skin disease on physical exam and skin pathology. METHODS: SSc patients completed the scleroderma modification of the Health Assessment Questionnaire (SHAQ), Skindex-29 and a Skin Symptom Assessment questionnaire developed for this study. Correlations were assessed between the Skin Symptom Assessment and SHAQ, Skindex-29, modified Rodnan skin score, and skin pathological features including myofibroblast staining completed on the same date. RESULTS: Tight, hard and rigid/stiff skin symptoms correlated moderately highly with the modified Rodnan skin score (r = 0.445, P = 0.0008; r = 0.486, P = 0.0002; and r = 0.488, P = 0.0002, respectively). Tight skin symptoms correlated moderately with myofibroblast infiltration (r = 0.544, P = 0.0023) and hyalinized collagen (r = 0.442, P = 0.0164), while both hard and rigid/stiff skin correlated moderately with inflammation (r = 0.401, P = 0.0310 and r = 0.513, P = 0.0045), myofibroblast infiltration(r = 0.480, P = 0.0084 and r = 0.527, P = 0.0033) and hyalinized collagen (r = 0.453, P = 0.0137 and r = 0.478, P = 0.0087), while the SHAQ was not found to correlate with any of these pathological changes. In contrast, painful skin symptoms correlated moderately with the SHAQ (r = 0.413, P = 0.0073), and with the three domains of Skindex-29: Symptoms, Emotions and Functioning. Skindex-29 indicates that dcSSc patient skin symptoms are nearly as severe as those of patients with psoriasis or atopic dermatitis. CONCLUSION: Patient reported skin symptoms correlate with clinical and pathological measures in the skin. A validated patient reported skin symptom instrument might considerably improve evaluation of SSc skin disease.

Occupational therapy integrated with a self-administered stretching program on systemic sclerosis patients with hand involvement.

OBJECTIVES: To evaluate the effect of occupational therapy (OT) intervention, integrated with a self-administered stretching program on the hands of patients with SSc, after one and three months of treatment. METHODS: We enrolled 31 patients with SSc, randomly allocated to the occupational group (15 patients) or to the control group (16 patients). Each patient received specific outcome measures: Canadian Occupational Performance Measure (COPM), HAQ, Short-Form Health Survey (SF-36), Duruoz Hand Index (DHI), reassessed after 1 (T1) and three months (T2). RESULTS: At T1 and T2 we found a statistically significant improvement from baseline values of COPM Performance and COPM Satisfaction in the OT group compared to baseline. At T2 HAQ values and Mental SF36 were also significantly improved. In the control group we found a statistically significant improvement of HAQ values and Mental SF36 at T1, confirmed at T2. COPM Performance was also significantly improved. The comparison between the two groups showed a greater improvement in the OT group concerning COPM Performance at T1 and T2. Mental SF-36 score greater improved in the control group at T1. CONCLUSIONS: Our results indicate that a rehabilitation program including OT and self-administered stretching exercises may be effective to improve and maintain hand function in patients with SSc.

Systemic sclerosis: rehabilitation as a tool to cope with disability.

In patients with systemic sclerosis (SSc), local disability of the hands and face, due to the involvement of skin, subcutaneous tissues and musculoskeletal system, is scarcely improved by pharmacological therapy, but may be treated efficaciously with rehabilitation, which can prevent and reduce local disability, thus ameliorating global disability and impaired Quality of Life, related to changes in the hands and face. In SSc, in order to be efficacious, rehabilitation should: 1. include and use both local treatments of hands and face and global rehabilitation techniques; 2. be different according to the different SSc phases and subsets; 3. include different techniques to tailor treatment to the personal needs and abilities of the patients.

Safety, tolerability and potential efficacy of injection of autologous adipose-derived stromal vascular fraction in the fingers of patients with systemic sclerosis: an open-label phase I trial.

BACKGROUND: In patients with systemic sclerosis (scleroderma, SSc), impaired hand function greatly contributes to disability and reduced quality of life, and is insufficiently relieved by currently available therapies. Adipose tissue-derived stromal vascular fraction (SVF) is increasingly recognised as an easily accessible source of regenerative cells with therapeutic potential in ischaemic or autoimmune diseases. We aimed to measure for the first time the safety, tolerability and potential efficacy of autologous SVF cells local injections in patients with SSc with hand disability. METHODS: We did an open-label, single arm, at one study site with 6-month follow-up among 12 female SSc patients with Cochin Hand Function Scale score >20/90. Autologous SVF was obtained from lipoaspirates, using an automated processing system, and subsequently injected into the subcutaneous tissue of each finger in contact with neurovascular pedicles. Primary outcome was the number and the severity of adverse events related to SVF-based therapy. Secondary endpoints were changes in hand disability and fibrosis, vascular manifestations, pain and quality of life from baseline to 2 and 6 months after cell therapy. FINDINGS: All enrolled patients had surgery, and there were no dropouts or patients lost to follow-up. No severe adverse events occurred during the procedure and follow-up. Four minor adverse events were reported and resolved spontaneously. A significant improvement in hand disability and pain, Raynaud's phenomenon, finger oedema and quality of life was observed. INTERPRETATION: This study outlines the safety of the autologous SVF cells injection in the hands of patients with SSc. Preliminary assessments at 6 months suggest potential efficacy needing confirmation in a randomised placebo-controlled trial on a larger population. FUNDING: GFRS (Groupe Francophone de Recherche sur la Sclérodermie). CLINICAL TRIALS NUMBER: NCT01813279.

The effect of a combined rehabilitation program on the temporomandibular joint in systemic sclerosis evaluated by ultrasound exam.

PURPOSE: Temporomandibular joint (TMJ) involvement is frequent in Systemic Sclerosis (SSc). Dysfunction and X-ray changes of TMJ were described only in few observational studies. Treatment as well has been seldom considered. Aim of the present study was to evaluate the effects on TMJ of two specifically designed physiotherapy protocols. METHODS: The study group included 26 SSc outpatients (22 females and 4 males with mean age ± SD 59.08 ± 10.31 years). Thirteen patients were randomly assigned to a treatment (protocol 1) including home exercises for TMJ and thirteen to a treatment (protocol 2) including home exercises and a combined procedure. The rehabilitation effects on the TMJ were evaluated by ultrasound examination (UE) in static and dynamic phases. UE was performed in all patients before and at the end of the treatment and after a follow up (8 weeks). RESULTS: Both rehabilitation protocols induced a significant improvement (protocol 1: p < 0.01 and protocol 2: p < 0.005) of mouth opening with a long-lasting effect. Protocol 2 was more effective than protocol 1. A significant increase of bilateral condyle-head temporal bone distance was detected by UE at the end of both treatments. It was maintained at follow-up in patients treated with Protocol 2. CONCLUSIONS: The present investigation shows that a rehabilitation program characterized by home exercises with a combined procedure is useful to recover the function of TMJ. The data also show that UE is helpful in the evaluation of TMJ in SSc and in the assessment of the efficacy of the rehabilitation programs.

Psychosocial aspects of systemic sclerosis.

PURPOSE OF REVIEW: In 2010, an international consortium of researchers published a consensus agenda for research on psychosocial aspects of systemic sclerosis (SSc; scleroderma). The present review summarizes recent research on SSc-related depression and anxiety, fatigue and sleep, pain, pruritus, body image distress, sexual function, work disability, healthcare needs, psychosocial interventions, and psychoneuroimmunology. RECENT FINDINGS: Researchers have used structured interviews to establish prevalence rates for clinical mood disorders in SSc, although anxiety remains understudied and distress may be a useful outcome to consider. Longitudinal research has identified predictors of fatigue. Research on body image distress suggests the importance of changes in the facial skin and hands. Studies have identified sexual function problems in women and men with SSc. A prospective study found that breathing problems and fatigue predicted workplace disability. A randomized controlled trial evaluating multidisciplinary care showed benefits for health-related quality of life. SUMMARY: There has been a recent expansion in studies of psychosocial aspects of SSc, and in the validation of psychosocial measures that can be used in clinics to identify problems and track outcomes; however, prospective studies remain scarce. To better address the psychosocial needs of persons with SSc, interventions need to be developed and tested via randomized controlled trials with power to detect clinically meaningful changes.

The Italian version of the Mouth Handicap in Systemic Sclerosis scale (MHISS) is valid, reliable and useful in assessing oral health-related quality of life (OHRQoL) in systemic sclerosis (SSc) patients.

In systemic sclerosis (SSc), mouth and face involvement leads to problems in oral health-related quality of life (OHRQoL). Mouth Handicap in Systemic Sclerosis scale (MHISS) is a 12-item questionnaire specifically quantifying mouth disability in SSc, organized in 3 subscales. Our aim was to validate Italian version of MHISS, by assessing its test-retest reliability and internal and external consistency in Italian SSc patients. Forty SSc patients (7 dSSc, 33 lSSc; age and disease duration: 57.27 ± 11.41, 9.4 ± 4.4 years; 22 with sicca syndrome) were evaluated with MHISS. MHISS was translated following a forward-backward translation procedure, with independent translations and counter-translation. Test-retest reliability was evaluated, comparing the results of two administrations, with intraclass correlation coefficient (ICC). Internal consistency was assessed by Cronbach's α and external consistency by comparison with mouth opening. MHISS has a good test-retest reliability (ICC: 0.93) and internal consistency (Cronbach's α:0.99). A good external consistency was confirmed by correlation with mouth opening (rho: -0,3869, p: 0.0137). Total MHISS score was 17.65 ± 5.20, with scores of subscale 1 (reduced mouth opening) of 6.60 ± 2.85 and scores of subscales 2 (sicca syndrome) and 3 (aesthetic concerns) of 7.82 ± 2.59 and 3.22 ± 1.14. Total and subscale 2 scores are higher in dSSc than in lSSc. This result may be due to the higher presence of sicca syndrome in dSSc than in lSSc (p = 0.0109). Our results support validity and reliability in Italian SSc patients of MHISS, specifically measuring SSc OHRQoL.

Rehabilitation Interventions in Systemic Sclerosis: A Systematic Review and Future Directions.

OBJECTIVE: To systematically review evidence of rehabilitation interventions for improving outcomes in systemic sclerosis (SSc) and to evaluate evidence quality. METHODS: Several electronic databases were searched to identify studies in which rehabilitation professionals delivered, supervised, or participated in interventions for individuals with SSc. Randomized controlled trials (RCTs) or non-randomized trials, one-arm trials, and prospective quasi-experimental studies with interventions were included if they had ≥10 participants. Quality appraisal was conducted by 2 independent raters using the Physiotherapy Evidence Database (PEDro) Scale. RESULTS: A total of 16 good or excellent quality studies (15 RCTs, 1 prospective quasi-experimental study) were included. Most rehabilitation interventions focused on hands/upper extremities, followed by multicomponent, orofacial, and directed self-management. Sample sizes varied between 20-267 participants (median 38). In 50% of studies, participants in intervention groups significantly improved compared to controls. Most studies demonstrated within-group improvements in intervention groups. Interventions varied in content, delivery, length, and dose and outcome measures collected. CONCLUSION: Existing evidence provides some support for rehabilitation in SSc, such as interventions that focus on hand and upper extremity outcomes or are multicomponent, although there is high study heterogeneity. The evidence base would benefit from interventions testing similar replicable components, use of common outcome measures, and incorporation of delivery modes that enable larger sample sizes. There are challenges in recruiting participants due to the rarity of SSc and high disease burden, as participants' involvement in rehabilitation studies requires active participation over time. Intervention studies designed to reduce participation barriers may facilitate translation of effective interventions into practice.