1481-P: Patients with Cystic Fibrosis–Related Diabetes Present with Specific Pancreatic Anomalies

Abstract

Cystic fibrosis related diabetes (CFRD) is found in 20 to 50% of adults with cystic fibrosis (CF). Repeated oral glucose tolerance tests are used to screen for CFRD, but are not perfect indicators and can be tiresome for patients who already undergo many medical tests. We hypothesized that pancreatic imaging may differ between patients with and without CFRD and that the patients with the more “at-risk” pancreatic morphology could be followed more closely with the aim to detect diabetes early. We performed a non-matched case-control retrospective study comparing the abdominal ultrasounds (U.S.) and computed tomography (CT) (including abdominal slices of thoracic CT) of patients with CF with or without CFRD (“CFRD patients” and “CF patients”, respectively). Forty-four CFRD patients and 58 CF patients were included. Thirty-seven percent of the U.S. results were either uninterpretable or discordant with the CT results. Therefore, only the results of the CT were eventually analyzed. Seventy-one percent of CFRD patients presented with complete pancreatic involution (CPI), versus 57% of CF patients (p = 0.0016), and 98% of CFRD patients presented with at least partial pancreatic involution. Patients with CPI presented more frequently with exocrine pancreatic insufficiency than patients with a normal CT scan (95% versus 29%, p< 0.0001). Unexpectedly, 22% of CFRD patients presented with pancreatic calcifications, versus none of the CF patients (p = 0.0003). Patients with pancreatic calcifications did not differ from patients without, except that they were less likely to be homozygous for DeltaF508 mutation (22 versus 53%, p = 0.04). CPI is more often seen in CFRD patients than in CF patients, but is still a very frequent occurrence. A more distinctive feature is the presence of pancreatic calcifications. These might be used as a warning signal to look more closely for diabetes in patients with CF, and can be searched for on abdominal slices of routinely performed thoracic CT. Disclosure L. Alexandre-Heymann: None. M. Puerto: Other Relationship; Self; Ipsen Biopharmaceuticals. P. Burgel: Advisory Panel; Self; AstraZeneca, Boehringer Ingelheim International GmbH, Chiesi USA, Inc., Vertex Pharmaceuticals. Consultant; Self; GlaxoSmithKline plc., Pfizer Inc., Teva Pharmaceutical Industries Ltd., Zambon. Speaker’s Bureau; Self; Novartis AG. E. Larger: None.