To review the most relevant extrapulmonary manifestations of cystic fibrosis (CF), and illustrate their imaging findings. To highlight that, although pulmonary manifestations may dominate the clinical course in CF, many other organs and systems can be involved.

Cystic fibrosis (CF) is an autosomal recessive disease caused by a great number of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The CFTR gene encodes an epithelial ion channel essential for absorption and secretion of salt and water in various organs and tissues such as the airways, pancreas, intestine, liver, sweat glands, vas deferens, among others, making CF a multisystemic condition. CF causes significant morbidity and mortality in the affected patients and is considered the most lethal inherited disease in the white...

Different imaging methods play an important role in the management of CF patients by presenting a set of imaging findings in the most commonly affected extrapulmonary locations. Head and neck manifestations: Almost every patient with CF develops chronic rhinosinusitis and shows radiological evidence of sinus disease. Computed tomography (CT) can depict sinus obstruction secondary to thick impacted mucus, mucocele formation, and bony sclerosis from chronic inflammation ( Fig. 1 ). Nasal polyps are frequently found on physical examination, but imaging can also display signs of...

As the survival of CF patients improves and pulmonary complications are better managed, extrapulmonary manifestations of the disease are becoming more important. The radiologist should be familiar with the spectrum of extrapulmonary manifestations in CF and their associated imaging findings, and promptly recognize the complications associated with this condition.

J. Rosa; Lisbon/PT - nothing to disclose P. Lameira; Lisbon/PT - nothing to disclose M. V. Roque; Lisbon/PT - nothing to disclose J. Fonseca-Santos; Lisbon/PT - nothing to disclose

Averill, S., Lubner, M., Menias, C., Bhalla, S., Mellnick, V., Kennedy, T. and Pickhardt, P. (2017) "Multisystem Imaging Findings of Cystic Fibrosis in Adults: Recognizing Typical and Atypical Patterns of Disease",American Journal of Roentgenology, 209(1), pp. 3-18. doi: 10.2214/ajr.16.17462. Oomen, K. and April, M. (2012) "Sinonasal Manifestations in Cystic Fibrosis",International Journal of Otolaryngology, 2012, pp. 1-7. doi: 10.1155/2012/789572. Gillespie, C., O’Reilly, M., Allen, G., McDermott, S., Chan, V. and Ridge, C. (2017) "Imaging the Abdominal Manifestations of Cystic Fibrosis",International Journal of Hepatology, 2017, pp. 1-9....