Abstract

BackgroundSystemic sclerosis (SSc) is a chronic disease characterized by autoimmunity, vasculopathy and fibrosis. During the disease course, patients with SSc are prone to accumulate multiple organ damage, increasing their vulnerability to adverse outcomes in comparison with individuals of the same age: a phenomenon called frailty. One of the most used definitions of frailty is the physical frailty phenotype (PFP) by Fried, et al. The PFP consists of 5 components: unintentional weight loss, exhaustion, muscle weakness, slow walking speed, and low physical activity. There is scarce data about frailty in patients with SSc.ObjectivesTo study the prevalence and clinical aspects of PFP in a sample of patients with SSc. Also, we aim to investigate the diagnostic accuracy of the Fatigue, Resistance, Ambulation, Illness and Loss of weight (FRAIL) scale and the Edmonton frailty scale (EFS) using the PFP as the reference standard.MethodsCross-sectional study, including patients with SSc according to the 2013 ACR-EULAR classification criteria or the criteria suggested by Le Roy and Medsger for early disease. PFP assessment was according to the original definition, except for physical activity domain, assessed with the International Physical Activity Questionnaire (IPAQ). Patients were classified according to Fried’s criteria in robust (0), pre-frail (1-2), and frail (≥3). FRAIL scale and EFS were also applied to the same individuals. The FRAIL score ranges from 0 to 5 and patients were classified as robust (0), pre-frail (1-2), or frail (≥3). The EFS score ranges from 0–17 and categorizes patients as not frail (0–4), vulnerable (5–6), mild frailty (7–8), moderate frailty (9–10), and severe frailty (≥11). For the diagnostic assessment of FRAIL scale and EFS, we estimated the area under the receiver operating characteristic curve (AUC), considering PFP as the reference standard and dichotomizing the results in frail vs. non-frail.ResultsBetween March and December 2019, 82 SSc patients were consecutively included. The mean age and disease duration were 60.4 (±10.6) and 13.4 (±8.2) years, respectively; 91.5% were women, and 19.5% with diffuse cutaneous SSc. The PFP distribution was: 8 (9.8%) robust, 47 (57.3%) pre-frail and 27 (32.9%) frail patients. The PFP domains´ frequencies were: low physical activity in 57 (69.5%), muscle weakness in 41 (50%), exhaustion in 34 (41.5%), unintentional weight loss in 15 (18.3%) and slow walking speed in 8 (9.8%) patients. Using the FRAIL scale, 44 (53.7%) patients were considered pre-frail and 25 (30.5%) frail. According to the EFS, 26 patients (31.7%) were classified as vulnerable and 12 (14.6%) as frail: mild in 6 (7.3%), moderate in 4 (4.9%) and severe in 2 (2.4%). The AUC against PFP was: 0.871 (95% CI 0.789-0.954, p<0.001) for FRAIL scale and 0.870 (95% CI 0.791-0.948, p<0.001) for EFS (Figure 1).ConclusionFrailty is prevalent in patients with long-standing SSc, and most of them are considered pre-frail or vulnerable. Low physical activity, muscle weakness and exhaustion are among the most frequent clinical aspects of the frailty phenotype. Both FRAIL scale and EFS showed overlapping diagnostic accuracy against PFP as the reference standard, and FRAIL scale seems to be more feasible than EFS.

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