Bacterial Cholangitis in Autosomal Dominant Polycystic Kidney and Liver Disease

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.

Pansomatostatin Agonist Pasireotide Long-Acting Release for Patients with Autosomal Dominant Polycystic Kidney or Liver Disease with Severe Liver Involvement

“…As polycystic liver disease (PLD) progresses, there is expansion of liver cysts and liver parenchyma. The enlarged liver compresses adjacent organs (heart, kidneys, spleen, diaphragm, gastrointestinal tract, and vasculature) and may lead to infectious complications (cholelithiasis, cyst infection, and cholangitis), which is debilitating in a subgroup (especially women) (3)(4)(5)(6)(7). Treatment interventions include cyst aspiration; fenestration or sclerotherapy; liver resection; octreotide; and, in severe cases, liver or combined liver-kidney transplantation (8)(9)(10).…”

Jaundice in Polycystic Liver Disease: More Than Meets the Eye

“…A recently published case series from the Mayo clinic described 29 patients with definite or suspected bacterial cholangitis in the setting of PLD with or without associated PKD 5 . Predictors of cholangitis included prior ERCP with sphincterotomy, cholelithiasis, or cholecystectomy prior to the first episode of cholangitis, duodenal diverticulum, or prior kidney transplantation.…”