Increased Occurrence of Pericardial Effusion in Patients with Autosomal Dominant Polycystic Kidney Disease

Abstract

Background and objectives: Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disease with prominent connective tissue manifestations. A frequent occurrence of asymptomatic pericardial effusion has been observed in patients with ADPKD.Design, setting, participants, & measurements: Consecutive computed tomography scans from 60 patients with ADPKD (group 1), 100 patients without ADPKD and with serum creatinine concentration of >1.1 mg/dl (group 2), and 100 potential kidney donors (group 3) were retrospectively examined. Pericardial effusion was graded from 0 to 4 on the basis of the distance between the parietal and visceral pericardia at mid, mid-low, and low levels of the heart on transaxial computed tomography scan sections.Results: Twenty-one (35%) of 60 patients in group 1 were found to have pericardial effusion, compared with 9 (9%) and 4 (4%) patients in groups 2 and 3, respectively. Ten of the 21 patients with pericardial effusion in group 1 but none of the patients in groups 2 and 3 had moderate to high effusion scores. The presence and severity of pericardial effusion were not associated with age, renal dysfunction, or hypertension. All pericardial effusions were asymptomatic.Conclusions: Pericardial effusion occurs with an increased frequency in patients with ADPKD, possibly as a result of increased compliance of the parietal pericardium. Although frequently moderate to large, these effusions are generally well tolerated and clinically inconsequential.

Sensory functions of motile cilia and implication for bronchiectasis

“…We found that 45.7% subjects (54 of 118) had pericardial effusions comparable to the 35% prevalence previously reported (64). There was no significant association between renal function, cardiac abnormalities or pericardial effusion and 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.…”

The ADPKD genespkd1a/bandpkd2regulate extracellular matrix formation

“…for understanding the fibrotic defects that are often associated with PKD (Cuppage et al, 1980;Wilson et al, 1992), as well as understanding other Pkd1 matrix-associated phenotypes, including intracranial aneurysm (Brooke et al, 2003;Ruigrok et al, 2005); vascular fragility and maintenance of the aortic wall ECM (Kim et al, 2000;Hassane et al, 2007); overlapping connective tissue disorders (Somlo et al, 1993;Kaplan et al, 1997); abdominal wall hernia (Morris-Stiff et al, 1997); and increased pericardial compliance associated with pericardial effusion in ADPKD patients (Qian et al, 2007). Our finding that a reduction in matrix gene expression by col2a1 knockdown can rescue polycystin axis curvature defects suggests that abnormalities in matrix composition or integrity are likely to be developmental defects linked directly to polycystin function, as opposed to secondary consequences of tissue damage or deformity.…”

“…Vascular defects are also observed in Pkd1 hypomorphic mouse mutants (Kim et al, 2000;Hassane et al, 2007), where basement membrane thickening is the earliest pathology associated with dissecting aneurysm (Hassane et al, 2007). Other extra renal manifestations of ADPKD that may involve matrix structural defects include gastrointestinal cysts, cardiac valvular defects and pericardial effusion (Hossack et al, 1988;Qian et al, 2007). An altered ECM has long been known to be associated with ADPKD pathology in human tissue (Candiano et al, 1992;Somlo et al, 1993), and in ADPKD animal and cell culture models (Schafer et al, 1994).…”