Cystic fibrosis related diabetes (CFRD) prognosis
Zahrae Sandouk1, Farah Khan2, Swapnil Khare3
1Internal Medicine, Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, MI, USA.
Insights
Cystic fibrosis related diabetes (CFRD) significantly worsens outcomes in cystic fibrosis (CF) patients, causing decreased lung function and increased mortality. Further research is needed to understand how new CFTR modulators impact CFRD prognosis and onset.
Area of Science:
- Endocrinology
- Pulmonology
- Metabolic diseases
Background:
- Cystic fibrosis related diabetes (CFRD) affects 40-50% of adult CF patients.
- Unlike other diabetes types, CFRD's primary impact is not micro/macrovascular disease, but worsened CF prognosis.
- Negative health impacts, including reduced lung function and nutritional status, begin before CFRD diagnosis.
Purpose of the Study:
- To review the specific complications and prognostic impacts of CFRD in cystic fibrosis.
- To explore the proposed mechanisms linking CFRD to adverse outcomes in CF.
- To consider the potential influence of emerging CFTR modulator therapies on CFRD.
Main Methods:
- Literature review and synthesis of existing research on CFRD and its complications.
- Analysis of proposed pathophysiological mechanisms.
- Discussion of the evolving landscape of CF treatment with CFTR modulators.
Main Results:
- CFRD is associated with decreased lung function, poor nutrition, and increased mortality in CF patients.
- Key mechanisms include insulin insufficiency leading to protein catabolism and hyperglycemia causing inflammation/infection.
- CFTR modulators are improving CF health, but their effect on CFRD is yet to be determined.
Conclusions:
- CFRD presents unique challenges in cystic fibrosis, distinct from other diabetes forms.
- Understanding CFRD's mechanisms is crucial for managing CF patient outcomes.
- The long-term impact of CFTR modulators on CFRD development and progression requires further investigation.
Abstract:
Cystic fibrosis related diabetes (CFRD) occurs in at least 40-50% of adults with CF. With other forms of diabetes, microvascular and macrovascular disease are the major causes of morbidity and mortality. Macrovascular disease is rare in CF. While microvascular disease does occur in this population, there are CF-specific diabetes complications that have a more important impact on prognosis. The additional diagnosis of diabetes in CF is associated with decreased lung function, poor nutritional status, and an overall increase in mortality from lung disease. These negative findings start even before the clinical diagnosis of CFRD, during the period when patients experience abnormal glucose tolerance related to insulin insufficiency. The main mechanisms by which CFRD negatively affects prognosis are thought to be a combination of 1) protein catabolism, decreased lean body mass and undernutrition resulting from insulin insufficiency, and 2) an increased pro-inflammatory and pro-infectious state related to intermittent hyperglycemia. With the introduction of CFTR modulators, the care of CF patients has been revolutionized and many aspects of CF health such as BMI and lung function are improving. The impact of these drugs on the adverse prognosis related to the diagnosis of diabetes in CF, as well as the potential to delay or prevent onset of CFRD remain to be determined.
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