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Bacterial Cholangitis in Autosomal Dominant Polycystic Kidney and Liver Disease

William P Martin1, Lisa E Vaughan2, Kotaro Yoshida3

  • 1Diabetes Complications Research Centre, Conway Institute of Biomolecular and Biomedical Research, School of Medicine, University College Dublin, Dublin, Republic of Ireland.

Mayo Clinic Proceedings. Innovations, Quality & Outcomes
|June 14, 2019
PubMed

Insights

Bacterial cholangitis is a complication of autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (ADPLD). Risk factors for cholangitis in ADPKD-associated polycystic liver disease include gallstones, prior cholecystectomy, and type 2 diabetes.

Area of Science:

  • Hepatology
  • Nephrology
  • Infectious Diseases

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (ADPLD) are genetic disorders characterized by cyst formation in the kidneys and liver, respectively.
  • Polycystic liver disease (PLD) can lead to complications such as bacterial cholangitis, an infection of the bile ducts.

Purpose of the Study:

  • To describe the characteristics of first episodes of bacterial cholangitis in patients with ADPKD and ADPLD.
  • To identify risk factors associated with cholangitis in patients with ADPKD-associated PLD.

Main Methods:

  • Retrospective review of electronic medical records from January 1, 1996, to June 30, 2017.
  • Identification and categorization of cholangitis cases (suspected or definite) by expert review.
  • Nested case-control study to determine risk factors for cholangitis in ADPKD patients.

Main Results:

  • Twenty-nine cases of cholangitis complicating PLD were identified (24 ADPKD-associated PLD, 5 ADPLD).
  • In ADPKD-associated PLD with definite cholangitis, significantly higher odds were observed for gallstones (OR 21.6), prior cholecystectomy (OR 12.2), duodenal diverticulum (OR 13.5), type 2 diabetes mellitus (OR 6.41), prior ERCP (OR 14.0), and prior kidney transplant (OR 8.06).

Conclusions:

  • Gallstones, prior cholecystectomy, duodenal diverticulosis, type 2 diabetes mellitus, prior endoscopic retrograde cholangiopancreatography (ERCP), and prior kidney transplant are significant risk factors for cholangitis in patients with ADPKD-associated PLD.
Abstract

Keywords:
ADPKD, autosomal dominant polycystic kidney diseaseADPLD, autosomal dominant polycystic liver diseaseALP, alkaline phosphataseALT, alanine aminotransferaseAST, aspartate aminotransferaseCT, computed tomographyERCP, endoscopic retrograde cholangiopancreatographyICD-10, International Classification of Diseases,Tenth RevisionICD-9, International Classification of Diseases,Ninth RevisionMCR, Mayo Clinic, Rochester, MNMRI, magnetic resonance imagingOR, odds ratioPET, positron emission tomographyPLD, polycystic liver diseaseT2DM, type 2 diabetes mellitus

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