马凡综合征患者疲劳的决定因素：一项使用患者报告结局测量信息系统（PROMS）的研究

Determinants of fatigue in patients with Marfan syndrome: a study using PROMS.

作者信息

Cavalier Ines, Kamga Leslie Toko, Morin Laurence, Bal Laurence, Faivre Laurence, Jondeau Guillaume, Tran Viet-Thi, Milleron Olivier

机构信息

Department of Epidemiology, Biostatistics and Clinical Research, Hôpital Bichat- Claude Bernard, INSERM CIC-EC 1425, AP-HP.Nord-Université Paris-Cité, Paris, France.

Centre d'Épidémiologie Clinique, Hôpital Hôtel-Dieu, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.

出版信息

Orphanet J Rare Dis. 2025 Jul 11;20(1):359. doi: 10.1186/s13023-025-03880-4.

DOI:10.1186/s13023-025-03880-4

PMID:40646614

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12255052/

Abstract

BACKGROUND

Fatigue is often reported by individuals with Marfan syndrome (MFS). However, the determinants of fatigue and its impact on the daily lives of patients with MFS remain poorly understood. We sought to assess the level of fatigue and its determinants in individuals with MFS.

METHODS

We conducted a cross-sectional study in ComPaRe Marfan, an e-cohort of MFS patients. Fatigue was assessed using the FACIT questionnaire already used in a wide range of chronic diseases. Pain and its interference with daily life were assessed using the Brief Pain Inventory. We performed univariate and multivariable linear regressions to identify determinants of fatigue.

RESULTS

A total of 162 people with MFS completed the FACIT-Fatigue questionnaire. The median age was 46 and 59% were women. The median FACIT-Fatigue score was 31(IQR 22-39) and over ¾ of the cohort had a FACIT-Fatigue score below 40 corresponding to at least some level of fatigue. In the multivariate model, only pain interference was associated with fatigue (Coeff= -0.34, CI95%: [-0.46; -0.22], p < 0.001). Beta-blocker treatment, history of aortic, lens or scoliosis surgery were not associated with FACIT fatigue score. In addition, the FACIT fatigue scores was repeated every month during 3 months and were stable over time.

CONCLUSION

Fatigue is common in patients with MFS and is associated with pain that interferes with daily life. Therefore, pain management in MFS patients could improve quality of life and fatigue.

摘要

背景

马凡综合征（MFS）患者常报告有疲劳症状。然而，疲劳的决定因素及其对MFS患者日常生活的影响仍知之甚少。我们旨在评估MFS患者的疲劳程度及其决定因素。

方法

我们在ComPaRe Marfan（一个MFS患者的电子队列）中进行了一项横断面研究。使用已在多种慢性疾病中使用的FACIT问卷评估疲劳。使用简明疼痛量表评估疼痛及其对日常生活的干扰。我们进行了单变量和多变量线性回归以确定疲劳的决定因素。

结果

共有162名MFS患者完成了FACIT-疲劳问卷。中位年龄为46岁，59%为女性。FACIT-疲劳评分的中位数为31（四分位间距22-39），超过四分之三的队列FACIT-疲劳评分低于40，这对应于至少某种程度的疲劳。在多变量模型中，只有疼痛干扰与疲劳相关（系数=-0.34，95%置信区间：[-0.46；-0.22]，p<0.001）。β受体阻滞剂治疗、主动脉、晶状体或脊柱侧弯手术史与FACIT疲劳评分无关。此外，FACIT疲劳评分在3个月内每月重复测量，且随时间稳定。