Mouth Handicap In Systemic Sclerosis Research Articles

Assessing the effect of PRP addition to facial micro-lipofilling for patients suffering from Scleroderma: A prospective routine care analysis

Combining fat graft with platelet derived products is now common practice in regenerative surgery. We proposed to assess the safety and efficacy of Platelet-Rich Plasma (PRP) addition to a micro-lipofilling protocol for facial treatment of patients suffering from Systemic Sclerosis (SSc). Main objective was to evaluate the improvement of the Mouth Handicap In Systemic Sclerosis (MHISS) scale score at 6 months post-therapy. Included SSc patients had a MHISS score equal or up to 20. Surgery was performed under general anesthesia. Micro-fat and PRP (CCA-NA from DEPA Classification) were mixed in a 70/30 ratio, before injection in peri-oral sites according to a specific protocol. Efficacy criteria were recorded at baseline, 3 and 6 months. Moreover, we compared this cohort (current study) to a former (2015) non-enriched micro-lipofilling cohort in the same indication, using the same protocol. Thirteen women patients with mean age of 53.2 years (±14.3) have been included. At baseline, mean MHISS score was 29.5 (±8.7) and significantly decreased to 22.5 (±7.8) at 6 months (P=0.016), corresponding to a 22.0% of improvement from baseline, with a mean decrease of 6.5 points (±7.5) at 6 months. Patients received a mean volume of 30.8ml PRP-micro-fat (±8.1ml). PRP addition appeared beneficial, however, controlled studies are required to determine its superiority to facial micro-lipofilling.

Efficacy of Fractional CO2 Laser for Improvement of Limited Mouth Opening in Systemic Sclerosis.

Systemic sclerosis is a multisystem auto-inflammatory disease-causing fibrosis in the skin and internal organs. A frequent complication of systemic sclerosis is the limited mouth opening (LMO), a difficult-to-treat condition with only a few treatment options available. The aim of this study was to evaluate the response of fractional carbon dioxide (CO2) laser resurfacing in LMO associated with systemic sclerosis. This was a hospital-based prospective study in which diagnosed cases of systemic sclerosis were taken. Patients who had significant LMO and who gave informed consent were included in the study. Fractional CO2 laser treatment was performed in the perioral area. Patients were assessed at baseline, after three and six sessions, and 3 months after the last session. Assessment was done by measurement of the interincisor distance (IID) using a ruler and calculation of the mouth handicap in systemic sclerosis (MHISS) scale. Improvement in IID occurred 3 months after the first session with a mean gain of +5 mm (range 2-7). At 6 months, a mean gain of +8.5 mm (range 7-10) in IID was observed (P < 0.001). The MHISS score decreased by a mean of 14 (range 11-17) (P < 0.001). All patients showed improvement of mouth opening, which allowed the patients to have better phonation and the patients were able to have proper dental care posttreatment in the form of brushing of teeth and other dental procedures. The adverse effects noted in these patients included erythema that resolved spontaneously or after icing posttreatment. Other adverse effects noted were stinging and burning sensations that were mild and transient. Fractional CO2 laser forms a safe, effective, and well-tolerated treatment modality for improvement of LMO in systemic sclerosis. The limitations of this study were less number of patients and no long-term follow-up.

Rehabilitation of the face and temporomandibular joint in systemic sclerosis.

Background:Systemic sclerosis (SSc) alterations of the face and of the mouth cause aesthetic modifications and disability, impairing self-esteem and quality of life (QoL). The aim of this study was to verify the effects of two rehabilitation protocols on facial mimic and mouth opening.Methods:A total of 47 SSc patients (40 females and 7 males, mean age ± SD 59.08 ± 10.31 years), were consecutively selected: 22 were randomly assigned to protocol 1 [home exercises for temporomandibular joint (TMJ), mimic, masticatory and cervical spine muscles] and 25 to protocol 2 (home exercises and combined physiotherapeutic procedures performed by a physiotherapist). Each treatment had a duration of 12 weeks with a follow up of 8 weeks. TMJ dysfunction, orofacial involvement, disability, QoL, and safety were assessed at enrollment (T0), at the end of the treatment (T1), and at follow up (T2).Results:Both Protocol 1 and Protocol 2 induced significant improvements of some clinical and clinimetric parameters, but better results were obtained with Protocol 2. In the comparison between the effects of Protocol 1 and Protocol 2 at T1 and T2, a significant difference was observed only for Mouth Handicap in SSc [MHISS; Total (p = 0.00178] and for MHISS Mouth opening (p = 0.0098) at T1. No significant difference of indices of short-form 36 was observed.Conclusion:The present data suggest that TMJ involvement in SSc may be managed by rehabilitation treatments. The action of a physiotherapist prescribing and personalizing exercises may induce better therapeutic effects.

The reliability and validity of the Arabic version of the Mouth Handicap in Systemic Sclerosis (MHISS) Questionnaire in Arabian systemic sclerosis patients

BackgroundSystemic sclerosis (SSc) is an autoimmune multi-systemic disease with predominant affection of connective tissue. Skin is characteristically affected in SSc and is almost responsible for oral complications as well as aesthetic changes which affect the patient’s oral health-related quality of life. The Mouth Handicap in Systemic Sclerosis (MHISS) Questionnaire is the first tool to subjectively calculate the handicap associated with mouth disability in SSc patients. This study aimed to test for reliability, the validity of the Arabic version of the MHISS to be used in Arabian systemic sclerosis.ResultsThis study was conducted on 38 females with SSc with a mean age of 48.7 ± 5.01 years and a mean disease duration of 6.23 ± 3.14 years—18 patients (47.4%) have diffuse SSc while 20 (52.6%) have localised SSc. The mean value of the total MHISS for SSc patients was 12.21 ± 3.51, test–retest reliability of the Arabic version was excellent (ICC = 0.991) with excellent internal consistency (Chronbach’s α = 0.892) with significant negative correlation with mouth opening (r = − 0.512, p < 0.05) and no correlation with the Health Assessment Questionnaire Disability Index (HAQ-DI).ConclusionOur results demonstrated that the Arabic version of the MHISS questionnaire had excellent test–retest reliability and very good validity in measuring mouth disabilities in Arabian SSc patients.

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.

Factors related to alexithymia in patients with systemic sclerosis: a tight relationship with facial image dissatisfaction.

To assess clinical and psychosocial factors related to alexithymia in systemic sclerosis (SSc). We enrolled 40 consecutive SSc patients in a cross-sectional study evaluating alexithymia with Toronto Alexithymia scale (TAS-20). We measured Beck Depression inventory (BDI), Hamilton Anxiety rating scale (HAM-H), 36-Items Short-Form Healthy Survey (SF-36), Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue, Visual Analog Scale (VAS) pain, Pittsburgh Sleep Quality Index (PSQI), Satisfaction with Appearance Scale (SWAP), and Mouth Handicap in Systemic Sclerosis (MHISS). The prevalence of alexithymia was 42%. Alexithymic patients presented increased depressive (p = ≤ 0.001) and anxiety symptoms (p = ≤ 0.001), sleep disorders (p = 0.03), pain (p = 0.02), esthetic concerns (p = 0.03), disability in activities (p = 0.03) and reduced scores of SF-36 in mental components summary (MCS) (p = ≤ 0.001) and physical components summary (PCS) (p = 0.01). We found significant correlations with sleep disorders (r = 0.41, p = ≤ 0.001), BID (r = 0.35, p = 0.04), facial image dissatisfaction (r = 0.35, p = 0.04), mouth disability (r = 0.51, p = 0.005), depressive (r = 0.6, p = ≤ 0.001), and anxiety symptoms (r = 0.48, p = ≤ 0.001), fatigue (r = - 0.45 p = 0.005), SF-36 PCS (r = - 0.51, p = ≤ 0.001) and MCS (r = - 0.65, p = ≤ 0.001). In multiple linear regression analysis, SWAP facial was the only variable associated with TAS-20 [0.99 (0.48) p = 0.05]. Alexithymia correlates with several psychosocial factors but seems strongly related to facial image dissatisfaction.

Autologous Fat Grafting in the Treatment of Facial Scleroderma.

Systemic sclerosis (SSc) is a rare systemic autoimmune disease, characterized by progressive cutaneous and internal organ fibrosis. Orofacial manifestations of systemic sclerosis are extremely disabling and treatment options are limited. In this study, we aimed to assess the safety and efficacy of autologous fat grafting in the face of patients with systemic sclerosis. We enrolled 16 SSc patients suffering from facial sclerosis and limited mouth opening capacity. Autologous fat injection ranging from 15 to 40 ml was administered per patient, based on their face morphology. The patients were evaluated at baseline and 3 months after fat injection. Evaluations included mouth opening capacity, mouth handicap in systemic sclerosis (MHISS), Rodnan skin sclerosis score, skin biophysical properties using a sensitive biometrologic device with the assessment of cutaneous resonance running time (CRRT), volumizing and aesthetic effects based on pre- and posttreatment photographs, possible side effects, and global patient satisfaction. Clinical assessment showed autologous fat transfer significantly improved mouth opening capacity and the MHISS and Rodnan score of patients with facial scleroderma (p value <.001). The aesthetic and/or functional results of fat injection were satisfying to about 80% of the patients. The changes in CRRT values were not significant. Our findings support the possible therapeutic role of autologous fat grafting in improving facial scleroderma both in aesthetic and in functional aspects. This trial is registered with IRCT20180209038677N1.

Changes over Time and Responsiveness of the Cochin Hand Function Scale and Mouth Handicap in Systemic Sclerosis Scale in Patients with Systemic Sclerosis: A Prospective Observational Study.

To assess changes over time and responsiveness of the Cochin Hand Function Scale (CHFS) and Mouth Handicap In Systemic Sclerosis (MHISS) scale in patients with systemic sclerosis (SSc). This was a prospective longitudinal study. Participants were drawn from a convenience sample of individuals with SSc, who were attending the annual Congress of the Association des Sclérodermiques de France, the French SSc patients' association. Participants were evaluated twice by the CHFS, MHISS scale, Health Assessment Questionnaire, McMaster-Toronto Arthritis Patient Preference Disability Questionnaire, and other outcome measures. Differences in measures over time were evaluated using the Wilcoxon signed rank test. Responsiveness was assessed by the effect size and standardized response mean. Nonparametric Spearman rank correlation (r) was used to assess correlation between outcome measures. Forty-nine patients were assessed. The CHFS and MHISS scores worsened over time (mean [SD] differences 2.7 [10.7], P = 0.08, and 3.5 [9.8], P = 0.14, respectively). The CHFS effect size and standardized response mean values were -0.16 and -0.24, respectively, and MHISS values were -0.53 and -0.52, respectively. The correlation between change in the CHFS score and change in Health Assessment Questionnaire and McMaster-Toronto Arthritis Patient Preference Disability Questionnaire scores was fair to moderate, whereas changes in MHISS score were poorly or not correlated to other measures. For patients considering their state deteriorated, the MHISS scale was the most responsive instrument. The CHFS and MHISS scores may be useful in detecting changes in location-specific limitations in activity for SSc patients.

Systematic review of systemic sclerosis-specific instruments for the EULAR Outcome Measures Library: An evolutional database model of validated patient-reported outcomes

The EULAR Outcome Measures Library (OML) is a freely available database of validated patient-reported outcomes (PROs). The aim of this study was to provide a comprehensive review of validated PROs specifically developed for systemic sclerosis (SSc) to feed the EULAR OML. A sensitive search was developed in Medline and Embase to identify all validation studies, cohort studies, reviews, or meta-analyses in which the objective were the development or validation of specific PROs evaluating organ involvement, disease activity or damage in SSc. A reviewer screened title and abstracts, selected the studies, and collected data concerning validation using ad hoc forms based on the COSMIN checklist. From 13,140 articles captured, 74 met the predefined criteria. After excluding two instruments as they were unavailable in English the selected 23 studies provided information on seven SSc-specific PROs on different SSc domains: burden of illness (symptom burden index), functional status (Scleroderma Assessment Questionnaire), functional ability (scleroderma Functional Score), Raynaud's phenomenon (Raynaud's condition score), mouth involvement (Mouth Handicap in SSc), gastro-intestinal involvement (University of California Los Angeles-Scleroderma Clinical Trial Consortium Gastro-Intestinal tract 2.0), and skin involvement (skin self-assessment). Each of them is partially validated and has different psychometric requirements. Seven SSc-specific PROs have a minimum validation and were included in the EULAR OML. Further development in the area of disease-specific PROs in SSc is warranted.

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, 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.

Differences in patients with systemic sclerosis recruited from associations and tertiary care settings

ObjectiveTo compare patients with systemic sclerosis (SSc) recruited from a patient association or a tertiary care setting. MethodsWe evaluated 248 SSc patients attending 4 annual meetings of a patient association between 2004 and 2007 (177) or during hospitalization (71). health-related quality of life (HRQoL) was assessed by the SF-36, global disability by the health assessment questionnaire (HAQ), hand disability by the Cochin Hand Function Scale (CHFS), mouth disability by the Mouth Handicap in Systemic Sclerosis (MHISS) scale, global hand and wrist mobility by the Kapandji index. ResultsAs compared with hospitalized patients, those from the patient association were significantly older (mean age 58.73±12.04 vs 53.818±13.1; p=0.001) and had a longer disease duration (10.98±8.7 vs 7.13±6.723 p=0.0001). Association patients had significantly increased disability of the hand (CHFS 21.8±19.8 vs 9.8±14.1; p=0.0001) and mouth (MHISS 20.65±10.8 vs 13.25±9.3; p=0.0001) and impaired hand and wrist mobility (Kapandji score 38.05±10.26 vs 43.90±8.26, p=0.001). The 2 groups did not differ in global disability or physical and mental scores of the SF36. ConclusionPatients recruited from a patient association have more severe SSc than do hospitalized patients. This finding must be taken into account in the design of surveys and clinical trials.

The rehabilitation of facial involvement in systemic sclerosis: efficacy of the combination of connective tissue massage, Kabat’s technique and kinesitherapy: a randomized controlled trial

In Systemic Sclerosis (SSc), face involvement causes functional loss as well as aesthetic changes and loss of the self-image. The aim of the work is to evaluate the efficacy of a rehabilitation program based on the combination of Kabat's technique, connective massage and kinesitherapy specifically conceived for the face of SSc patients. Forty SSc patients were enrolled: 20 patients (interventional group) were treated for 9 weeks (twice a week, 1 h per session) with a combined connective tissue massage, Kabat's technique, kinesitherapy and home exercise program, and 20 patients (control group) were assigned only home exercise program. All patients were assessed at baseline (T0), at the end of the treatment (T1) and after 9 weeks of follow-up (T2). They were evaluated with SF-36, HAQ, modified Rodnan skin score, mouth opening in centimeters and Mouth Handicap in Systemic Sclerosis (MHISS) scale. At T1, both groups improved in mouth opening (P < 0.05), but the improvement was maintained at T2 only in interventional group. In interventional group, facial skin score ameliorated at T1 and maintained at T2 (P < 0.05 vs. T0), while no change was observed in controls. In both groups, SF-36 and HAQ were not affected by the treatment. MHISS scale improved significantly in interventional group at T1 (P < 0.001), while no change was found in controls. The combination of connective tissue massage, Kabat's technique, kinesitherapy and home-based exercises is more effective than a home exercise program alone in the rehabilitative treatment of SSc facial involvement.

Patient preference disability questionnaire in systemic sclerosis: A cross‐sectional survey

To assess patient priorities concerning disability in systemic sclerosis (SSc). A total of 150 SSc patients (22 men) fulfilling the American College of Rheumatology and/or LeRoy and Medsger criteria for SSc were evaluated by the McMaster Toronto Arthritis Patient Preference Disability Questionnaire (MACTAR), Karnofsky performance status (KPS), Cochin Hand Function Scale, Health Assessment Questionnaire (HAQ), Hospital Anxiety and Depression Scale, Mouth Handicap in SSc (MHISS) scale, and global perception regarding their health status. Correlations between scores were analyzed using Spearman's coefficient. Logistic regression analysis was used to determine factors associated with patients' global perception of their health. Of the patients investigated, 81 (54%) had limited cutaneous SSc, 65 (43.3%) diffuse SSc, and 4 (2.7%) limited SSc. The 3 disability domains most often cited were walking (82 patients [54.6%]), housekeeping (67 patients [44.6%]), and sport activities (59 patients [39.3%]). The MACTAR score correlated moderately with KPS (r = 0.58) but only weakly with the HAQ score (r = 0.38). In multivariate analysis, 2 factors were associated with patients' negative global perception of their health status: KPS (odds ratio [OR] 1.07, 95% confidence interval [95% CI] 1.00-1.15) and MHISS score (OR 0.93, 95% CI 0.88-0.99). For assessing SSc patient priorities concerning disability, the MACTAR has acceptable construct validity. Its weak correlation with the HAQ suggests that it adds useful information on disability.

Development and validation of a scale for mouth handicap in systemic sclerosis: the Mouth Handicap in Systemic Sclerosis scale

Objective:To develop and assess the reliability and construct validity of a scale assessing disability involving the mouth in systemic sclerosis (SSc).Methods:We generated a 34-item provisional scale from mailed responses of...