Cystic Fibrosis-Related Diabetes (CFRD)

Abstract

Cystic fibrosis-related diabetes (CFRD) is the most frequent comorbidity in CF. The prevalence is age-dependent and abnormalities in/of glucose homeostasis start early in life. As CFRD has an impact on pulmonary function and life expectancy, early diagnosis and treatment is mandatory. Screening is needed because initially, most patients with CFRD do not show any typical symptoms of diabetes. The question of which screening method gets the best results is still under discussion. For treatment insulin is recommended but a relevant percentage of patients do not use it, and even if insulin is used, there is no consensus on what the best insulin regime in the case of CFRD is. Recently, oral antidiabetic drugs were shown to be as effective and safe as insulin in the initial treatment of CFRD. This treatment might reduce the additional treatment burden for patients with CFRD. The best way to monitor CFRD is also under discussion (HbA1c and/or continuous glucose monitoring; CGM). The threshold of HbA1c might be lower than for other types of diabetes. As patients with CF become older, the duration of CFRD will also increase and typical diabetes complications will occur. So far, these are mainly microvascular complications. The new CFTR modulators might influence not only pulmonary function but potentially also glucose homeostasis.