Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes

Abstract

ystic fibrosis-related diabetes (CFRD) is the most common comorbidity in people with cystic fibrosis (CF), occurring in 20% of adolescents and 40 -50% of adults (1). While it shares features of type 1 and type 2 diabetes, CFRD is a distinct clinical entity. It is primarily caused by insulin insufficiency, although fluctuating levels of insulin resistance related to acute and chronic illness also play a role. The additional diagnosis of CFRD has a negative impact on pulmonary function and survival in CF, and this risk disproportionately affects women (2-4). In contrast to patients with other types of diabetes, there are no documented cases of death from atherosclerotic vascular disease in patients with CFRD, despite the fact that some now live into their sixth and seventh decades.