Coexisting cystic lung disease as a rare extra-renal manifestation of autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) classically presents with multiple bilateral renal cysts and ultimately progresses to end stage renal disease. While many of the extra-renal manifestations of ADPKD are well-documented, associated pulmonary findings are particularly rare, having only been recently been reported in a handful of studies to date. A 69-year-old female with ADPKD presented to our hospital with respiratory complaints. High resolution computed tomography revealed bronchiectasis, cystic lung disease, and interstitial fibrosis. The patient did not have concurrent risk factors or coexisting disease processes to explain the etiology of her airway and cystic lung disease, which we suggest are manifestations of ADPKD. We have not found a previous report of interstitial lung disease in this setting.