Evaluation of quality of life, functionality and disability in patients with systemic sclerosis in a University Hospital

Introduction: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by progressive fibrosis of the skin and internal organs that promotes high morbidity and mortality. Objective: To evaluate the functionality, disability and quality of life of patients with systemic sclerosis and to compare the clinical forms of the disease. Methods: Cross-sectional, descriptive and analytical study performed at the Rheumatology Clinic of the Hospital das Clínicas of the Federal University of Pernambuco (HC-UFPE) from August 2018 to April 2019. The non-probabilistic, convenience sample consisted of 60 patients diagnosed with systemic sclerosis (SSc), followed at the Rheumatology outpatient clinic of the Hospital das Clínicas, Federal University of Pernambuco. To evaluate the outcomes, the following instruments were used: Cochin Hand Functional Scale (CHFS) for hand function; 12-Item ShortForm Health Survey (SF-12) for quality of life; and Scleroderma Health Assessment Questionnaire (SHAQ) for functionality and disability. Results: The mean results for CHFS, SHAQ, SF-12 Physical Component Summary and SF-12 Mental Component Summary were 14.5 (6.0-29.75), 1.01±0.56, 35.04±8.09, 40.94±10.56, respectively. There were no significant differences in CHFS outcomes between patients with diffuse and limited forms of SSc, SHAQ and the mental component of SF-12. However, in the physical component of SF-12, a better score was found in patients with the diffuse form of the disease (p=0.04). Conclusion: Patients with SSc present an important impairment of hand function, quality of life and functional capacity, and those with limited cutaneous form present worse scores of the physical component in the evaluation of quality of life.


INTRODUCTION
Systemic sclerosis (SSc) is an autoimmune disease of unknown etiology, which is characterized by excess collagen synthesis, leading to fibrosis of the skin and organs 1 .
It is a rare disease, with a prevalence of approximately 10 cases per 100,000 inhabitants, Silva AM, Roque LCSC, Gonçalves RSG, Duarte ALBP, Tenório AS, Dantas AT ABCS Health Sci. 2020;45: e020025 and its incidence is higher in females 2,3 . There are two clinical forms of SSc classification, according to the extent of cutaneous involvement: limited cutaneous and diffuse cutaneous. In the limited cutaneous there is a thickening of the skin at the extremities (face, forearm and hands, legs and feet) and in the diffuse cutaneous the most proximal regions of the limbs, trunk and abdomen are affected 2 . The involvement of organs is relatively minor in the limited cutaneous form 4 .
Regarding muscle changes, the presence of myopathy is described in between 20% and 86% of patients, arthralgia in 23% to 81% and synovitis in 15% to 20% 5 . The range of motion may be decreased, causing a wave of contractures whose prevalence evaluated by physical examination varies from 24% to 56% 5 .
Therefore, individuals with SSc can show loss of mobility and decrease of muscle strength, as well as contractures and deformities of the hands. All of these changes may cause significant impairment in functionality and quality of life, and cause an increase in overall disability, impairing daily activities 6,7 .

METHODS
The present study is characterized as a cross-sectional, de- Clinical data of patients were obtained from medical records.
Lung fibrosis was defined using high-resolution computed tomography (HRCT); esophagus involvement was determined by scintilography or endoscopy; proximal muscle weakness and elevated serum creatine kinase indicated muscle involvement were recorded. Pulmonary artery pressure was estimated by Doppler echocardiogram; pressures above 35 mmHg were interpreted as pulmonary hypertension.
In order to evaluate the functionality, the Cochin Hand Functional Scale (CHFS) was used, a questionnaire developed in France and validated in Portuguese that aims to evaluate the functionality of the hands and has been applied in patients 8,10 with SSc. It consists in 18 questions that cover manual functional activities, whose answers range from 0 (patient who performs the activity without difficulty) to 5 (patient who is unable to do the activity). The final score is obtained by adding the results of all items 11,12 .
Quality of life was evaluated through the 12-item Short- (the higher the result, the better the SF-12) 13 . It was applied to evaluate SF-12 in several diseases 14,15 and was previously applied to SSc 16 .

RESULTS
This study had a total of 60 patients, most of whom were female  Table 1.
The results regarding the evaluation of the functionality, disability and quality of life are described in Table 2 There was no association between the CHFS, SHAQ and SF-12 scores and the variables of age, time of diagnosis and the presence of specific clinical manifestations of the disease (Table 3). Similarly, associations between the respective scores were not observed (Table 4).

DISCUSSION
The present study demonstrated that patients with SSc have low hand function, quality of life and functional capacity. The