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. 2019 May 27;3(2):149-159.
doi: 10.1016/j.mayocpiqo.2019.03.004. eCollection 2019 Jun.

Bacterial Cholangitis in Autosomal Dominant Polycystic Kidney and Liver Disease

William P Martin  1 , Lisa E Vaughan  2 , Kotaro Yoshida  3 , Naoki Takahashi  3 , Marie E Edwards  4 , Andrew Metzger  4 , Sarah R Senum  4 , Tetyana V Masyuk  5 , Nicholas F LaRusso  5 , Matthew D Griffin  6 , Ziad El-Zoghby  4 , Peter C Harris  4 , Walter K Kremers  2 , David M Nagorney  7 , Patrick S Kamath  5 , Vicente E Torres  4 , Marie C Hogan  4
Affiliations

Bacterial Cholangitis in Autosomal Dominant Polycystic Kidney and Liver Disease

William P Martin et al. Mayo Clin Proc Innov Qual Outcomes. .

Abstract

Objective: To describe first episodes of bacterial cholangitis complicating autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (ADPLD) and to identify risk factors for cholangitis episodes among patients with ADPKD-associated polycystic liver disease (PLD).

Patients and methods: We searched the electronic medical records at our tertiary referral center for episodes of cholangitis in patients with ADPKD or ADPLD from January 1, 1996, through June 30, 2017. Cases were categorized as suspected or definite cholangitis by expert review. Clinical, laboratory, and radiologic data were manually abstracted. A nested case-control study was conducted to investigate risk factors for cholangitis in patients with ADPKD.

Results: We identified 29 cases of definite or suspected cholangitis complicating PLD (24 with ADPKD-associated PLD and 5 with ADPLD). Among patients with definite cholangitis in ADPKD-associated PLD (n=19) vs ADPLD (n=4), the mean ± SD age was 62.4±12.2 vs 55.1±8.6 years, and 9 (47.4%) vs 0 (0%), respectively, were male. The odds of gallstones (odds ratio [OR], 21.6; 95% CI, 3.17-927; P<.001), prior cholecystectomy (OR, 12.2; 95% CI, 1.59-552; P=.008), duodenal diverticulum (OR, 13.5; 95% CI, 2.44 to not estimable; P=.004), type 2 diabetes mellitus (OR, 6.41; 95% CI, 1.01 to not estimable; P=.05), prior endoscopic retrograde cholangiopancreatography (OR, 14.0; 95% CI, 1.80-631; P=.005), and prior kidney transplant (OR, 8.06; 95% CI, 1.72-76.0; P=.004) were higher in patients with ADPKD-associated PLD with definite cholangitis compared to controls.

Conclusion: Gallstones, prior cholecystectomy, duodenal diverticulosis, type 2 diabetes mellitus, prior endoscopic retrograde cholangiopancreatography, and prior kidney transplant constituted risk factors for cholangitis among patients with ADPKD-associated PLD.

Keywords: ADPKD, autosomal dominant polycystic kidney disease; ADPLD, autosomal dominant polycystic liver disease; ALP, alkaline phosphatase; ALT, alanine aminotransferase; AST, aspartate aminotransferase; CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; ICD-10, International Classification of Diseases,Tenth Revision; ICD-9, International Classification of Diseases,Ninth Revision; MCR, Mayo Clinic, Rochester, MN; MRI, magnetic resonance imaging; OR, odds ratio; PET, positron emission tomography; PLD, polycystic liver disease; T2DM, type 2 diabetes mellitus.

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Figures

Figure
Figure
Overview of cholangitis cases occurring in patients with polycystic liver disease (PLD). ADPKD = autosomal dominant polycystic kidney disease; ADPLD = autosomal dominant polycystic liver disease.
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