Respiratory Unit, University of Milano Bicocca, S. Gerardo Hospital, ASST Monza, Monza, Italy.
Division of Pulmonary Medicine, Department of Medicine, University of Alberta, 3-134 Clinical Sciences Building, 11304 83 Ave., Edmonton, AB, T6G 2G3, Canada.
Respir Res. 2021 Apr 17;22(1):109. doi: 10.1186/s12931-021-01711-1.
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive disorder with an estimated median survival time of 3-5 years after diagnosis. This condition occurs primarily in elderly subjects, and epidemiological studies suggest that the main risk factors, ageing and exposure to cigarette smoke, are associated with both pulmonary and extrapulmonary comorbidities (defined as the occurrence of two or more disorders in a single individual). Ageing and senescence, through interactions with environmental factors, may contribute to the pathogenesis of IPF by various mechanisms, causing lung epithelium damage and increasing the resistance of myofibroblasts to apoptosis, eventually resulting in extracellular matrix accumulation and pulmonary fibrosis. As a paradigm, syndromes featuring short telomeres represent archetypal premature ageing syndromes and are often associated with pulmonary fibrosis. The pathophysiological features induced by ageing and senescence in patients with IPF may translate to pulmonary and extrapulmonary features, including emphysema, pulmonary hypertension, lung cancer, coronary artery disease, gastro-oesophageal reflux, diabetes mellitus and many other chronic diseases, which may lead to substantial negative consequences in terms of various outcome parameters in IPF. Therefore, the careful diagnosis and treatment of comorbidities may represent an outstanding chance to improve quality of life and survival, and it is necessary to contemplate all possible management options for IPF, including early identification and treatment of comorbidities.
特发性肺纤维化（IPF）是一种慢性进行性疾病，诊断后中位生存时间估计为 3-5 年。这种情况主要发生在老年患者中，流行病学研究表明，主要危险因素，即衰老和吸烟，与肺部和肺外合并症（定义为单个个体中发生两种或多种疾病）有关。衰老和衰老通过与环境因素相互作用，可能通过多种机制导致 IPF 的发病机制，导致肺上皮细胞损伤并增加肌成纤维细胞对细胞凋亡的抵抗力，最终导致细胞外基质积累和肺纤维化。作为一个范例，端粒短的综合征代表典型的早衰综合征，通常与肺纤维化有关。IPF 患者衰老和衰老引起的病理生理特征可能转化为肺部和肺外特征，包括肺气肿、肺动脉高压、肺癌、冠状动脉疾病、胃食管反流、糖尿病和许多其他慢性疾病，这可能会导致 IPF 在各种结果参数方面产生重大负面影响。因此，仔细诊断和治疗合并症可能是改善生活质量和生存的绝佳机会，有必要考虑 IPF 的所有可能治疗方案，包括早期识别和治疗合并症。
