Moua Teng, Zand Ladan, Hartman Robert P, Hartman Thomas E, Qin Dingxin, Peikert Tobias, Qian Qi
Division of Pulmonary/Critical Care, Mayo Clinic Rochester, Rochester, Minnesota, United States of America.
Division of Nephrology, Mayo Clinic Rochester, Rochester, Minnesota, United States of America.
PLoS One. 2014 Apr 18;9(4):e93674. doi: 10.1371/journal.pone.0093674. eCollection 2014.
Polycystin 1 and 2, the protein abnormalities associated with autosomal dominant polycystic kidney disease (ADPKD), are also found in airway cilia and smooth muscle cells. There is evidence of increased radiologic bronchiectasis associated with ADPKD, though the clinical and functional implications of this association are unknown. We hypothesized an increased prevalence of both radiologic and clinical bronchiectasis is associated with APDKD as compared to non-ADPKD chronic kidney disease (CKD) controls.
A retrospective case-control study was performed at our institution involving consecutive ADPKD and non-ADPKD chronic kidney disease (CKD) patients seen over a 13 year period with both chest CT and PFT. CTs were independently reviewed by two blinded thoracic radiologists. Manually collected clinical data included symptoms, smoker status, transplant history, and PFT findings.
Ninety-two ADPKD and 95 non-ADPKD CKD control patients were compared. Increased prevalence of radiologic bronchiectasis, predominantly mild lower lobe disease, was found in ADPKD patients compared to CKD control (19 vs. 9%, P = 0.032, OR 2.49 (CI 1.1-5.8)). After adjustment for covariates, ADPKD was associated with increased risk of radiologic bronchiectasis (OR 2.78 (CI 1.16-7.12)). Symptomatic bronchiectasis occurred in approximately a third of ADPKD patients with radiologic disease. Smoking was associated with increased radiologic bronchiectasis in ADPKD patients (OR 3.59, CI 1.23-12.1).
Radiological bronchiectasis is increased in patients with ADPKD particularly those with smoking history as compared to non-ADPKD CKD controls. A third of such patients have symptomatic disease. Bronchiectasis should be considered in the differential in ADPKD patients with respiratory symptoms and smoking history.
常染色体显性遗传性多囊肾病（ADPKD）相关的蛋白质异常——多囊蛋白1和2，也存在于气道纤毛和平滑肌细胞中。有证据表明，ADPKD与放射学上支气管扩张的增加有关，尽管这种关联的临床和功能意义尚不清楚。我们假设，与非ADPKD慢性肾脏病（CKD）对照组相比，ADPKD患者中放射学和临床支气管扩张的患病率均有所增加。
在我们机构进行了一项回顾性病例对照研究，纳入了13年间连续就诊的ADPKD和非ADPKD慢性肾脏病（CKD）患者，均进行了胸部CT和肺功能检查（PFT）。CT由两名不知情的胸科放射科医生独立评估。人工收集的临床数据包括症状、吸烟状况、移植史和肺功能检查结果。
对92例ADPKD患者和95例非ADPKD CKD对照患者进行了比较。与CKD对照组相比，ADPKD患者中放射学支气管扩张的患病率增加，主要为轻度下叶病变（19%对9%，P = 0.032，比值比2.49（可信区间1.1 - 5.8））。在对协变量进行调整后，ADPKD与放射学支气管扩张风险增加相关（比值比2.78（可信区间1.16 - 7.12））。约三分之一有放射学病变的ADPKD患者出现症状性支气管扩张。吸烟与ADPKD患者放射学支气管扩张增加相关（比值比3.59，可信区间1.23 - 12.1）。
与非ADPKD CKD对照组相比，ADPKD患者尤其是有吸烟史的患者放射学支气管扩张增加。这类患者中有三分之一有症状性疾病。对于有呼吸道症状和吸烟史的ADPKD患者，鉴别诊断时应考虑支气管扩张。
