Cystic fibrosis related diabetes (CFRD) in the era of modulators: A scoping review

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 mod-ulator 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 con-cerns. 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.& COPY; 2022 Elsevier Ltd. All rights reserved.

Automated summary

Cystic fibrosis-related diabetes (CFRD) is a common complication of CF that increases with age. Poor glycemic control negatively affects lung function and weight, leading to higher risk of pulmonary exacerbations. The emergence of highly effective modulator therapies (HEMT) has improved the lives of CF patients, but their impact on CFRD is not well understood. This review examines the pathophysiology of CFRD, summarizes available evidence on HEMT impact, and highlights the research needs in this field.