Scleroderma (Limited and Diffuse)

This study aims to investigate the effectiveness of BETY, an exercise approach based on the biopsychosocial model, in children and adults diagnosed with scleroderma.

Physical functionality and performance are important for individuals diagnosed with scleroderma. There is a need to address children and adults diagnosed with scleroderma comprehensively and to evaluate them on a biopsychosocial basis to support their disease management. In light of all this literature, the aim was to evaluate the biopsychosocial characteristics of both pediatric and adult scleroderma patients and to examine their physical performance and functionality. Additionally, this study aims to identify effective tests that can be used in future research to assess physical performance and functionality in individuals diagnosed with scleroderma.

The Patient Self-Assessment of Skin Thickness in Upper Limb (PASTUL) questionnaire was developed to enable patients to assess skin thickness in systemic sclerosis (SSc) physically and has been validated for reliability and validity, as it correlates with the modified Rodnan Skin Score (mRSS), which rheumatologists assess. Thus, it has been demonstrated to be an applicable and easy approach for assessing skin thickness in SSc patients through patient-reported measurements. This study aimed to develop a Turkish version of the PASTUL questionnaire and to provide for its use in the Turkish population.

It is recommended to focus on the physical functionality, chewing, and swallowing performance of individuals diagnosed with scleroderma. There is a need to address children and adults diagnosed with scleroderma comprehensively and to evaluate them on a biopsychosocial basis to support their disease management. The aim was to assess the biopsychosocial characteristics of both children and adults diagnosed with scleroderma and to examine their chewing and swallowing performance. Additionally, this study aims to identify effective scales that can be used in future research to assess chewing and swallowing in individuals diagnosed with scleroderma.

Identify rare variants in candidate genes and pathways identified in familial SSc, in patients with sporadic SSc. Perform (spatial) transcriptomic and proteomic analyses of affected skin from patients with and without cutaneous fibrosis, for the patterns and levels of expression/activation of candidate genes and pathways. Test for dysregulation of expression/activation of candidate genes and pathways in live cells isolated from the blood and skin biopsy of patients, and for the impact of these dysregulations on cell appearance, behavior and function.

The aim of this study is to investigate the effects of IMT on respiratory muscle strength, pulmonary function tests, quality of life aerobic capacity, dyspnea, fatigue and physical activity level in scleroderma patients with ILD. The study will include 30 Scleroderma + ILD patients who are under follow-up in Fırat University Rheumatology Department. Patients will be randomly divided into two groups. High intensity intermittent inspiratory muscle training (H-IMT) will be applied to the respiratory muscle training group in addition to the exercise program. Respiratory muscle strength will be assessed by maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP); aerobic capacity will be assessed by exercise test and VO2max. Dyspnea will be assessed with the Modified Medical Research Council Scale; fatigue with the Fatigue Severity Scale; quality of life with the Scleroderma Health Assessment Questionnaire; physical activity level with the International Physical Activity Scale - Short Form