Regard L, Lafoeste H, Martin C, Chassagnon G, Burgel P-R
Université Paris Descartes, Sorbonne Paris cité, 75006 Paris, France; Service de pneumologie, hôpital Cochin, AP-HP, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France.
Université Paris Descartes, Sorbonne Paris cité, 75006 Paris, France; Service d'imagerie médicale, hôpital Cochin, AP-HP, 75014 Paris, France.
Rev Pneumol Clin. 2018 Oct;74(5):279-291. doi: 10.1016/j.pneumo.2018.09.012. Epub 2018 Oct 11.
Cystic fibrosis (CF) is a genetic disease with pulmonary involvement being predominant and often leading to respiratory failure and premature death. Non-pulmonary complications related to CF transmembrane conductance regulator (CFTR) defect are numerous and account for significant morbidity (exocrine pancreatic insufficiency, diabetes, CF-related liver disease, chronic sinusitis, osteoporosis). Improvement in patients' care led to a continuous increase in life expectancy, with a subsequent increase in the number of adult CF patients worldwide. Increased life expectancy comes with increased prevalence of CF-related comorbidities, but also with new emerging complications directly related to ageing (chronic kidney disease, cardiovascular risk factors, cancers). CFTR modulators might also contribute to modify the face of CF epidemiology and prognosis. Ageing with CF has become a challenge for CF patients and caregivers. This review summarizes classic and emerging comorbidities in the context of current growth and ageing of the CF population. It also addresses potential roles of CFTR modulators.
囊性纤维化（CF）是一种遗传性疾病，肺部受累最为突出，常导致呼吸衰竭和过早死亡。与囊性纤维化跨膜传导调节因子（CFTR）缺陷相关的非肺部并发症众多，是导致显著发病的原因（外分泌性胰腺功能不全、糖尿病、CF相关肝病、慢性鼻窦炎、骨质疏松症）。患者护理的改善使预期寿命持续增加，全球成年CF患者数量随之增加。预期寿命的增加伴随着CF相关合并症患病率的上升，同时也出现了与衰老直接相关的新并发症（慢性肾脏病、心血管危险因素、癌症）。CFTR调节剂也可能有助于改变CF的流行病学和预后情况。对于CF患者及其护理人员而言，伴随CF的衰老已成为一项挑战。本综述总结了在CF人群当前增长和老龄化背景下的经典和新出现的合并症。它还探讨了CFTR调节剂的潜在作用。
