Pneumology Unit, Centro Hospital e Universitário de Coimbra, Coimbra, Portugal.
Centre of Pneumology, Faculty of Medicine, University of Coimbra, Coimbra, Portugal.
Ther Adv Respir Dis. 2020 Jan-Dec;14:1753466620910092. doi: 10.1177/1753466620910092.
Idiopathic pulmonary fibrosis (IPF) is characterized by progressive parenchymal scarring, leading to dyspnoea, respiratory failure and premature death. Although IPF is confined to the lungs, the importance of IPF comorbidities such as pulmonary hypertension and ischaemic heart disease, lung cancer, emphysema/chronic obstructive pulmonary disease, gastroesophageal reflux, sleep apnoea and depression has been increasingly recognized. These comorbidities may be associated with increased mortality and significant loss of quality of life, so their identification and management are vital. The development of good-quality biomarkers could lead to numerous gains in the management of these patients. Biomarkers can be used for the identification of predisposed individuals, early diagnosis, assessment of prognosis, selection of best treatment and assessment of response to treatment. However, the role of biomarkers for IPF comorbidities is still quite limited, and mostly based on evidence coming from populations without IPF. The future development of new biomarker studies could be informed by those that have been studied independently for each of these conditions. For now, clinicians should be mostly attentive to clinical manifestations of IPF comorbidities, and use validated diagnostic methods for diagnosis. As research on biomarkers of most common diseases continues, it is expected that useful biomarkers are developed for these diseases and then validated for IPF populations.
特发性肺纤维化(IPF)的特征是进行性实质瘢痕形成,导致呼吸困难、呼吸衰竭和过早死亡。尽管 IPF 局限于肺部,但 IPF 合并症的重要性,如肺动脉高压和缺血性心脏病、肺癌、肺气肿/慢性阻塞性肺疾病、胃食管反流、睡眠呼吸暂停和抑郁,已越来越受到重视。这些合并症可能与死亡率增加和生活质量显著下降有关,因此识别和管理这些合并症至关重要。高质量生物标志物的开发可能会为这些患者的管理带来诸多益处。生物标志物可用于识别易感个体、早期诊断、评估预后、选择最佳治疗方法和评估治疗反应。然而,生物标志物在 IPF 合并症中的作用仍然相当有限,并且主要基于来自无 IPF 人群的证据。未来新的生物标志物研究的发展可以从针对这些疾病的独立研究中获得启示。目前,临床医生应主要关注 IPF 合并症的临床表现,并使用经过验证的诊断方法进行诊断。随着对大多数常见疾病的生物标志物研究的继续,预计这些疾病将开发出有用的生物标志物,并随后在 IPF 人群中进行验证。