Cystic fibrosis-related diabetes (CFRD) is a common complication of CF that increases in incidence as patients age. Poor glycemic control has been shown to negatively impact lung function and weight, resulting in higher risk of recurrent pulmonary exacerbations. With the advent of highly effective modulator therapies (HEMT), patients with CF are living longer and healthier lives. Consequently, CFRD and its microvascular complications are rising in prominence, becoming one of the most urgent clinical concerns. As HEMT were developed with the primary focus of improving pulmonary outcomes, it is not clear from the original phase III studies what the short- or long-term benefits of modulators might be on CFRD development and trajectory. In this review, we will examine the pathophysiology of CFRD, summarize and synthesize the available evidence of HEMT impact on CFRD and describe the emerging research needs in this field.

囊性纤维化相关糖尿病 (CFRD) 是 CF 的常见并发症，其发病率随着患者年龄的增长而增加。血糖控制不佳已被证明会对肺功能和体重产生负面影响，导致肺部病情复发的风险更高。随着高效调节剂疗法 (HEMT) 的出现，CF 患者的寿命更长、更健康。因此，CFRD 及其微血管并发症日益突出，成为最紧迫的临床问题之一。由于 HEMT 的开发主要目的是改善肺部结局，因此从最初的 III 期研究中尚不清楚调节剂对 CFRD 发展和轨迹的短期或长期益处是什么。在这篇综述中，我们将研究 CFRD 的病理生理学，