Department of Internal Medicine, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Rio Grande do Sul, Brazil; Division of Rheumatology, Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, Rio Grande do Sul, Brazil.
Department of Internal Medicine, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Rio Grande do Sul, Brazil.
Semin Arthritis Rheum. 2022 Oct;56:152077. doi: 10.1016/j.semarthrit.2022.152077. Epub 2022 Jul 28.
Systemic sclerosis (SSc) is a chronic disease characterized by autoimmunity, vasculopathy and fibrosis of several organs, such as skin, lungs, and heart. During the disease course, patients with SSc are prone to accumulating multiple organ damage and increasing their vulnerability to adverse outcomes. This increased vulnerability to adverse outcomes when exposed to a stressor among people of the same age is known as frailty. One of the most used definitions of frailty is the physical frailty phenotype (PFP), including 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.
To determine the prevalence and clinical profile of PFP in a sample of patients with SSc. To investigate the diagnostic accuracy of the Fatigue, Resistance, Ambulation, Illness and Loss of weight (FRAIL) scale, Edmonton frailty scale (EFS) and Short Physical Performance Battery (SPPB) using the PFP as the reference standard.
Cross-sectional study including 94 patients with SSc according to the 2013 ACR-EULAR classification criteria or the criteria suggested by Le Roy and Medsger for early disease. Gastrointestinal symptoms were assessed by the UCLA GIT 2.0 questionnaire, malnutrition was defined according to European Society of Clinical Nutrition and Metabolism (ESPEN) recommendations, and physical performance was assessed by SPPB. PFP assessment was according to the original definition, except for physical activity domain, assessed with the International Physical Activity Questionnaire (IPAQ). FRAIL scale and EFS were also applied to the same individuals. For diagnostic assessment of FRAIL, EFS and SPPB, 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.
According to PFP, 33 patients (35.1%) were considered frail and 53 patients (56.4%) pre-frail. According to FRAIL scale, 27 patients (28.7%) were considered frail and 53 patients (56.4%) pre-frail. According to EFS, 28 patients (29.7%) were classified as vulnerable and 15 (15.9%) as frail: mild in 8 (8.5%), moderate in 5 (5.3%) and severe in 2 (2.1%). According to SPPB, 19 patients (20.2%) were considered frail. The AUC against PFP was: 0.829 (95% CI 0.743-0.916) for FRAIL scale, 0.859 (95% CI 0.784-0.934) for EFS and 0.791 (95% CI 0.697-0.885) for SPPB. The PFP was associated with current use of glucocorticoids (p=0.011), UCLA GIT 2.0 score (p=0.001), HAQ (p<0.0001), patient and physician-assigned VAS (p<0.0001, both), malnutrition (p=0.007), hospitalizations in the past year (p=0.008) and dependence on BADL and IADL (p=0.027 and p<0.0001, respectively). The PFP was not associated with gender (p=0.679), age (p=0.303), disease duration (p=0.504), Rodnan skin score (p=0.918), diffuse subtype (p=0.116), polypharmacy (p=845) and sarcopenia (p=0.328).
Frailty is prevalent in patients with long-standing SSc and is associated with disability, limitations in daily activities and hospitalizations in the past year. Also, malnutrition and more severe gastrointestinal symptoms were more common in frail patients. Both FRAIL scale and EFS showed excellent diagnostic accuracy against PFP as the reference standard, however the FRAIL scale presents a higher sensitivity and seems to be more feasible and practical than EFS and SPPB in clinical practice.
系统性硬化症（SSc）是一种以自身免疫、血管病变和多个器官纤维化为特征的慢性疾病，如皮肤、肺和心脏。在疾病过程中，SSc 患者容易发生多种器官损伤，并增加不良结局的易感性。当处于相同年龄的人群中，由于压力源而导致的这种不良结局的易感性增加被称为脆弱。脆弱性的最常用定义之一是身体脆弱表型（PFP），包括 5 个组成部分：非有意体重减轻、疲惫、肌肉无力、步行速度缓慢和体力活动减少。关于 SSc 患者的脆弱性数据很少。
确定 SSc 患者样本中 PFP 的患病率和临床特征。使用 PFP 作为参考标准，调查疲劳、抵抗、活动、疾病和体重减轻（FRAIL）量表、埃德蒙顿脆弱性量表（EFS）和简短身体表现测试（SPPB）的诊断准确性。
横断面研究包括 94 名根据 2013 年 ACR-EULAR 分类标准或 Le Roy 和 Medsger 早期疾病标准诊断的 SSc 患者。使用 UCLA GIT 2.0 问卷评估胃肠道症状，根据欧洲临床营养与代谢学会（ESPEN）建议定义营养不良，使用 SPPB 评估身体表现。PFP 评估根据原始定义进行，除了体力活动域，使用国际体力活动问卷（IPAQ）进行评估。还对同一人群应用 FRAIL 量表和 EFS。为了评估 FRAIL、EFS 和 SPPB 的诊断准确性，我们估计了接受者操作特征曲线（ROC）的曲线下面积（AUC），将 PFP 作为参考标准，并将结果分为脆弱和非脆弱。
根据 PFP，33 名患者（35.1%）被认为是脆弱的，53 名患者（56.4%）是前脆弱的。根据 FRAIL 量表，27 名患者（28.7%）被认为是脆弱的，53 名患者（56.4%）是前脆弱的。根据 EFS，28 名患者被归类为脆弱，15 名患者（15.9%）为脆弱：轻度 8 名（8.5%），中度 5 名（5.3%），严重 2 名（2.1%）。根据 SPPB，19 名患者（20.2%）被认为是脆弱的。FRAIL 量表、EFS 和 SPPB 对 PFP 的 AUC 分别为：0.829（95%CI 0.743-0.916）、0.859（95%CI 0.784-0.934）和 0.791（95%CI 0.697-0.885）。PFP 与当前使用糖皮质激素（p=0.011）、UCLA GIT 2.0 评分（p=0.001）、HAQ（p<0.0001）、患者和医生分配的 VAS（p<0.0001，均）、营养不良（p=0.007）、过去一年的住院（p=0.008）以及对 BADL 和 IADL 的依赖（p=0.027 和 p<0.0001，分别）相关。PFP 与性别（p=0.679）、年龄（p=0.303）、疾病持续时间（p=0.504）、罗登皮肤评分（p=0.918）、弥漫性亚型（p=0.116）、多药治疗（p=845）和肌少症（p=0.328）无关。
长期 SSc 患者的脆弱性很常见，与残疾、日常活动受限和过去一年的住院有关。此外，脆弱患者更常见营养不良和更严重的胃肠道症状。FRAIL 量表和 EFS 对 PFP 作为参考标准均具有出色的诊断准确性，然而，FRAIL 量表具有更高的敏感性，并且在临床实践中似乎比 EFS 和 SPPB 更可行和实用。
