No abstract

Systemic sclerosis (SSc) is a multisystemic disorder characterized by functional and structural abnormalities of small blood vessels and fibrosis of the skin and internal organs. Eighty percent of subjects with SSc have orofacial manifestations. No tests for oral manifestations have been validated for this pathology, and in the literature there are few studies of speech therapy for subjects with SSc. These facts suggested the need for an exhaustive assessment of mouth mobility and muscle strength, and also of swallowing and voice, in order to plan a targeted and effective speech therapy. The Scleroderma Logopedic Scale (SLS) has been developed to assess disorders in five domains: Impairment, Swallow, Voice, Multifield, and Quality of Life. Perception of these disorders was assessed in 84 subjects with SSc and in 40 healthy subjects. After the first draft, a shorter form (39 items) was obtained after statistical analysis. This scale showed good discriminant and concurrent validity. Internal consistency was good: three of five subscales had a Cronbach alpha coefficient higher than 0.8. The test/retest coefficient for the total score was 0.94. Thirty-six percent of examined subjects showed moderate to severe oropharyngolaryngeal disorders. Swallowing disorders and impairment of mouth (e.g., decrease in mobility and strength) were the most commonly reported problems. Conversely, the change of voice due to the pathology was not perceived as a problem. Fifty-five percent of subjects reported a decreased level of quality of life.

The aim of the present study was to provide quantitative data of oral function in healthy subjects (HSs), validity of measurements and estimation of measurement bias, as well as quantify oral impairment in persons with scleroderma (SSc). 151 HSs and 12 subjects with SSc were recruited and assessed using instrumented tools, measuring maximal mouth opening; lip strength; and tongue strength, protrusion, retraction, and endurance. Twenty HSs were also retested 3-5 weeks later in order to assess the test-retest reliability of the measurements. Intraclass correlation coefficients proved to be satisfactory (>0.8) for both inter-rater and test-retest reliabilities of all measurements except for tongue retraction. In the HS group, maximal mouth opening and tongue and lips strength values were larger (P < 0.05) for males than females, while no significant differences were found for other variables. Older subjects had statistically significantly lower tongue retraction values and tongue endurance values than younger subjects. The SSc group showed a statistically significant decrease (P < 0.05) in almost all the measurements. Assessment procedures proved to be valid and reliable. Gender and height were predictors of mouth opening, lip and tongue strength, while age correlates with tongue retraction and endurance. Measurements highlighted the strong impact of SSc on oral functions and in particular on tongue protrusion, tongue strength, and endurance.

The aim of this study was to develop an exercise protocol to improve maximal mouth opening (MMO), tongue protrusion (Tprot), tongue strength (Tstren), and lip strength (Lstren), and to assess its effects on subjects with scleroderma. Methods: We performed four replicated single-system studies in a consecutive sample of subjects with scleroderma. An instrumented assessment measured MMO, Tprot, Tstren, and Lstren. Each day, subjects were assessed and performed orofacial exercises conducted by speech therapists. Treatments were first aimed at improving mouth physical characteristics by impairment-oriented exercises and then to improve skills with function-oriented exercises. Results: The mean phase differences between assessment and treatment phases across subjects were from 0.88 to 9.56 mm in MMO, from 2.03 to 12.3 mm in Tprot, from-0.12 to 5.35 N in Tstren, and from-0.84 to 5.19 N in Lstren. After treatment, 3 subjects crossed the 5th percentile discriminating normal from abnormal performances for both Tstren and Tprot, while this occurred in 2 subjects for MMO and Lstren. Conclusions: The results of this study suggest that rehabilitation appears to be useful in reducing tongue and lip impairments and in improving oral functions in subjects with scleroderma.