Determinants of fatigue in patients with Marfan syndrome: a study using PROMS
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. Method...
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BMC
2025-07-01
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| Series: | Orphanet Journal of Rare Diseases |
| Online Access: | https://doi.org/10.1186/s13023-025-03880-4 |
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| author | Ines Cavalier Leslie Toko Kamga Laurence Morin Laurence Bal Laurence Faivre Guillaume Jondeau Viet-Thi Tran Olivier Milleron |
| author_facet | Ines Cavalier Leslie Toko Kamga Laurence Morin Laurence Bal Laurence Faivre Guillaume Jondeau Viet-Thi Tran Olivier Milleron |
| author_sort | Ines Cavalier |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-9fae3b701c094d1fa6ac0fb04157bf16 |
| institution | DOAJ |
| issn | 1750-1172 |
| language | English |
| publishDate | 2025-07-01 |
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| series | Orphanet Journal of Rare Diseases |
| spelling | doaj-art-9fae3b701c094d1fa6ac0fb04157bf162025-08-20T03:06:05ZengBMCOrphanet Journal of Rare Diseases1750-11722025-07-012011310.1186/s13023-025-03880-4Determinants of fatigue in patients with Marfan syndrome: a study using PROMSInes Cavalier0Leslie Toko Kamga1Laurence Morin2Laurence Bal3Laurence Faivre4Guillaume Jondeau5Viet-Thi Tran6Olivier Milleron7Department of Epidemiology, Biostatistics and Clinical Research, Hôpital Bichat- Claude Bernard, INSERM CIC-EC 1425, AP-HP.Nord-Université Paris-CitéCentre d’Épidémiologie Clinique, Hôpital Hôtel-Dieu, Assistance Publique-Hôpitaux de Paris (AP-HP)Association MarfansCRMR Marfan et Apparentés, Centre Aorte Timone, CHU Timone, Aix–Marseille Université Centre de Génétique et Centre de Référence Anomalies du Développement et Syndromes Malformatifs FHU TRANSLAD, INSERM UMR1231 Hôpital d’Enfants CHU DijonCentre national de référence pour le syndrome de Marfan et pathologies apparentés Assistance Publique Hôpitaux de Paris, Service de Cardiologie, Hôpital Bichat ParisCentre d’Épidémiologie Clinique, Hôpital Hôtel-Dieu, Assistance Publique-Hôpitaux de Paris (AP-HP)Centre national de référence pour le syndrome de Marfan et pathologies apparentés Assistance Publique Hôpitaux de Paris, Service de Cardiologie, Hôpital Bichat ParisAbstract 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.https://doi.org/10.1186/s13023-025-03880-4 |
| spellingShingle | Ines Cavalier Leslie Toko Kamga Laurence Morin Laurence Bal Laurence Faivre Guillaume Jondeau Viet-Thi Tran Olivier Milleron Determinants of fatigue in patients with Marfan syndrome: a study using PROMS Orphanet Journal of Rare Diseases |
| title | Determinants of fatigue in patients with Marfan syndrome: a study using PROMS |
| title_full | Determinants of fatigue in patients with Marfan syndrome: a study using PROMS |
| title_fullStr | Determinants of fatigue in patients with Marfan syndrome: a study using PROMS |
| title_full_unstemmed | Determinants of fatigue in patients with Marfan syndrome: a study using PROMS |
| title_short | Determinants of fatigue in patients with Marfan syndrome: a study using PROMS |
| title_sort | determinants of fatigue in patients with marfan syndrome a study using proms |
| url | https://doi.org/10.1186/s13023-025-03880-4 |
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