rehabilitation program in patients with scleroderma

Conditions: Scleroderma; System; Sclerosis; Muscle Strain; Physical Disability; Quality of Life; Exercise

Summary: scleroderma (or systemic sclerosis - ssc) is one of the most neglected diseases worldwide, according to the world health organization. in the adult population with ss, the systemic effects of the disease, such as respiratory and peripheral muscle dysfunction, cause a decrease in quality of life. as a consequence, there is a concern about functional rehabilitation, since the aging of this population is already a reality. thus, the objective of this project is to evaluate the effects of functional rehabilitation on functional capacity and quality of life in women over 18 years of ss. in this longitudinal intervention study, patients will be submitted to a three-month rehabilitation program. before and after the intervention, patients will be submitted to the following assessments: cochin hand functional scale (chfs), short-form 36 health survey (sf-36); scleroderma health assessment questionnaire disability index (shaq-di); lung function; lung ultrasound; handgrip; glittre activities of daily living test. thus, it is expected that patients with ss will benefit significantly, with a consequent improvement in musculoskeletal function and , functional capacity and health-related quality of life.

in the current epidemiological context, chronic diseases affect very specific and neglected populations. in addition to their rarity, these diseases are characterized by a progressive increase in the life expectancy of their patients and important functional limitations due to their multisystemic character. in this context, there is systemic sclerosis - ssc (or scleroderma), which presents peculiar clinical and functional aspects that limit the activities of daily living (adl) of patients. although the focus of ssc treatment is the clinical control of the disease, a new perspective must be given to the aspect of functional rehabilitation. therefore, this study proposes a therapist-oriented home rehabilitation program (tohr) for these patients. considering that in ssc the hands are predominantly affected, and that functional incapacity of the hands is the main component of global incapacity in this population, the adl-glittre test has been used as a submaximal functional capacity test as it involves lower and upper limbs, as well as diversified tasks, coming closer to the activities that the individual does in its usual environment. thus, the present study aims to evaluate the effects of a tohr program on the functional capacity of patients with ssc, through the adl-glittre test, considering the impact of hand function, peripheral muscle strength, pulmonary function and quality of life. for this, women with ssc will be evaluated, submitted to the following tests: cochin hand functional scale, short-form 36 health survey; scleroderma health assessment questionnaire disability index; handgrip dynamometry; impulse oscillometry technique; spirometry; lung ultrasound and adl-glittre test. additionally, patients will undergo a 12-week tohr protocol. at the end of this period, all evaluations will be repeated. this will be a prospective, longitudinal study, with quantitative assessment of sample data. the aim of the study is to support the applicability of a tohr program in women with ssc.

Eligibility criteria:
inclusion criteria: * women with scleroderma, older than 18 years * clinical stability * signature of the informed consent term (tcle) exclusion criteria: * patients with inability to perform the glittre activities of daily living test * patients who have cognitive impairment by mini mental state examination (meen). * abandonment of treatment of scleroderma during the application of the protocol. * uncontrolled hypertension (\> 180/100 mmhg with medication use) * use of psychotropic drugs * any significant limitations due to osteoarthropathy * history of surgery in the previous six months with exercise restriction * ipaq with very active classification

Keywords: scleroderma physical therapy rehabilitation functional capacity exercise adl-glittre test