Multisystem Imaging Findings of Cystic Fibrosis in Adults: Recognizing Typical and Atypical Patterns of Disease.
AJR Am J Roentgenol. 2017 Jul;209(1):3-18. doi: 10.2214/AJR.16.17462. Averill S1, Lubner MG1, Menias CO2, Bhalla S3, Mellnick VM3, Kennedy TA1, Pickhardt PJ1.
OBJECTIVE: There is an expanding and increasingly heterogeneous population of adult patients with cystic fibrosis (CF). Although CF is usually diagnosed in children with progressive multisystem involvement, up to 7% of CF cases are currently diagnosed de novo in adults with subtle manifestations distinct from the typical features of classic CF. The purpose of this article is to present the wide spectrum of CF in adults, including both classic and nonclassic variants, with an emphasis on the nonclassic imaging findings.
CONCLUSION: Recurrent pancreatitis, chronic sinusitis, and congenital bilateral absence of the vas deferens (CBAVD) are several of the ways in which CF is identified in adult patients with relatively rare mutations and with overall milder manifestations. It is important for radiologists to recognize the wide spectrum of CF to optimally monitor disease progression and response to therapeutic interventions in distinct adult patient populations.