Création et validation d’un outil d’évaluation du handicap lié à l’atteinte du visage de la sclérodermie systémique

Abstract

Many studies have recorded significant impairment of health-related quality of life in systemic sclerosis patients using validated scales. However, these instruments are not specifically designed for facial signs. To develop and validate a specific questionnaire to assess the burden on patients with facial signs of systemic sclerosis and which we have named "Burden of Face Affected" (BoFA). BoFA was developed using standard methodology in 3phases: exploration, development and validation. In all, 197patients completed questionnaires. We analysed the degree of internal consistency (Cronbach's α) and external validity between BoFA and the 12-Item Short Form Healthy Survey (SF-12), the Mouth Handicap In Systemic Sclerosis Scale (MHISS), Rosenberg's self-esteem scale, and the Perceived Stress Scale (PSS). To assess reproducibility, a test-retest analysis was conducted. The original French version was translated into English and underwent cultural validation. The questionnaire comprises 20items grouped into 4dimensions. BoFA showed good internal consistency (Cronbach's α: 0.93). External validity was demonstrated in terms of good correlation between BoFA and other questionnaires, in particular MHISS (r=0.54). The test-retest analysis demonstrated good reproducibility (0.92). The BoFA score varied significantly according to the severity of facial scleroderma as assessed by the patients themselves. Facial involvement in systemic sclerosis may be considered by physicians to be a minor consequence of the disease and is often overlooked. Nevertheless, it is crucial for patients' quality of life. A number of studies have assessed the impact of facial signs on health-related quality of life using instruments such as DLQI (Dermatology Life Quality Index), SWAP (Satisfaction With Appearance Scale), Brief SWAP and SSPRO (Scleroderma Skin Patient-Reported Outcome). However, these are not specific for facial signs and focus on other sites. BoFA has good reliability and construct validity, and it assesses disability specifically involving the face in patients with systemic sclerosis. To our knowledge, BoFA is the first specific tool for assessing burden in patients with facial scleroderma. It is an easy-to-use tool for evaluating the burden of facial signs and may also be used to assess the degree of burden before and after treatment.