Autosomal Dominant Polycystic Kidney Disease Is Associated With an Increased Prevalence of Radiographic Bronchiectasis

Characterization of Primary Cilia in Human Airway Smooth Muscle Cells

“…22,35 Patients with ADPKD also exhibit prominent connective tissue abnormalities, 38 including abnormal arterial remodeling, intracranial aneurysms, hernia/diverticula, and dilation of the pericardium with pericardial effusion. 39 Recent studies 40,41 also show an approximately threefold increase in the occurrence of bronchiectasis in patients with ADPKD. Moreover, the bronchiectasis is frequently coupled with irregular bronchial wall thickness, without evidence of recurrent airway infection, inferring airway wall defects and possible ASM involvement.…”

Deletion of airway cilia results in noninflammatory bronchiectasis and hyperreactive airways

“…The impetus for these studies was the clinical observation that patients with PKD have an increased frequency of bronchiectasis (9). This finding led us to hypothesize that there is a link between cilia and the development of bronchiectasis.…”

“…Recently, an association between bronchiectasis and polycystic kidney disease (PKD) in humans has been reported (9). PKD is a "ciliopathic" disorder that involves the mutation of proteins that are necessary for the structure and function of nonmotile cilia in renal epithelial cells (4).…”

RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies

“…For example, recent reports have highlighted the function of BBS proteins in airway respiratory cilia (Shah et al, 2008). Studies in human patients with polycystic kidney diseases have noted a high incidence of bronchiectasis, a previously uncharacterized manifestation of this disease (Driscoll et al, 2008). In addition, some patients with PCD also have retinitis pigmentosa or kidney failure (Bonneau et al, 1993;Moore et al, 2006;Osman et al, 1991).…”