Sorbonne Université, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, F-75005, Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale du Département R3S, F-75013, Paris, France.
CHU Lille, Clinique des Maladies Respiratoires, F-59000 Lille, Inserm U1019, CNRS UMR 8204, Center for Infection and Immunity of Lille, Institut Pasteur de Lille, F 59000, Lille, France.
Respir Med. 2018 Dec;145:1-7. doi: 10.1016/j.rmed.2018.10.010. Epub 2018 Oct 17.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder leading to chronic respiratory failure. Few studies have investigated ALS-related dyspnoea, and none have characterised the emotional distress it inflicts. We hypothesised that ALS-related dyspnoea has a strong affective component that relates to quality of life.
This prospective, observational study was conducted in 41 ALS patients >18 with chronic respiratory failure and an indication for noninvasive ventilation (NIV). Dyspnoea was assessed using the Multidimensional Dyspnea Profile (MDP) at baseline and 1 month after NIV initiation. Correlations between scores evaluating the sensory and affective dimensions of dyspnoea and other patient-reported outcomes and pulmonary function tests were analysed.
Dyspnoea was described as intense (median [IQR] score on a 0-10 scale: 6.5 [4.0-7.5]). The sensory dimension of dyspnoea was polymorphic, but «air hunger» was the most common (48.8%) and the most intense (6 [4-8]) sensory descriptor. In the affective domain, most patients rated «anxious» (85.4%) and «afraid» (60.9%) above 0. The MDP affective dimension correlated significantly with other patient-reported outcomes, with the strongest correlation being between MDP «anxious» and the anxiety component of the Hospital Anxiety Depression Scale (Pearson's R = 0.70). One month after initiation of NIV, dyspnoea during unassisted breathing was described in virtually the same terms, particularly the affective dimension.
ALS-related dyspnoea is intense and fear-provoking, persists during unassisted breathing between NIV sessions, and significantly impacts health-related quality of life. This study highlights the need for increased awareness of and research into ALS-related dyspnoea.
肌萎缩侧索硬化症（ALS）是一种导致慢性呼吸衰竭的神经退行性疾病。很少有研究调查与 ALS 相关的呼吸困难，也没有研究描述其引发的情绪困扰。我们假设与 ALS 相关的呼吸困难具有强烈的情感成分，与生活质量有关。
这项前瞻性、观察性研究纳入了 41 名年龄>18 岁且患有慢性呼吸衰竭并需要无创通气（NIV）的 ALS 患者。在基线和开始 NIV 后 1 个月使用多维呼吸困难量表（MDP）评估呼吸困难。分析评估呼吸困难感觉和情感维度的评分与其他患者报告的结局和肺功能测试之间的相关性。
呼吸困难被描述为强烈（0-10 分制的中位数[IQR]评分：6.5 [4.0-7.5]）。呼吸困难的感觉维度呈多态性，但“空气饥饿”是最常见的（48.8%）和最强烈的（6 [4-8]）感觉描述符。在情感领域，大多数患者将“焦虑”（85.4%）和“恐惧”（60.9%）评为>0。MDP 情感维度与其他患者报告的结局显著相关，与医院焦虑抑郁量表（HADS）的焦虑成分之间的相关性最强（Pearson's R=0.70）。开始 NIV 后 1 个月，在无辅助呼吸期间，呼吸困难的描述几乎相同，特别是情感维度。
与 ALS 相关的呼吸困难强烈且令人恐惧，在 NIV 治疗期间的无辅助呼吸之间持续存在，并且对健康相关的生活质量有显著影响。这项研究强调了需要提高对与 ALS 相关的呼吸困难的认识和研究。
