Liver Graft Failure and Bile Cast Nephropathy

Abstract

The consequences of graft failure after liver transplantation (LT) range far beyond the liver. The kidneys are often affected, where persistent and progressive cholestasis can result in acute kidney injury (AKI) leading to the development of bile cast nephropathy (BCN). BCN is an often unrecognized condition that is characterized by proximal tubulopathy and the formation of bile casts in the distal tubules, which is almost diagnosed exclusively on a kidney biopsy or autopsy. This condition is potentially reversible, provided the bilirubin levels can be reduced early. LT may represent a treatment option in the case of irreversible liver (or liver graft) failure, which is beneficial for both the liver and the kidney. This paper reports a case of BCN in a patient with idiopathic graft failure after LT. Despite his chronic kidney disease, liver re-transplantation led to the successful improvement of his AKI.

Keywords: Acute kidney injury; Cholestasis; Hyperbilirubinemia; Liver failure; Liver transplantation.

Fig. 1

Clinical course of liver allograft failure with the development of bile cast nephropathy. The graph shows the bilirubin (red) and creatinine (blue) values in three different periods; 1) baseline period (after the first liver transplant), 2) period of allograft failure with major events; kidney biopsy, plasma exchange (five sessions) and liver re-transplantation with early post-transplant period and 3) long-term follow-up (>1 year after liver re-transplantation). LT, liver transplantation; FU, follow-up.

Fig. 2

Bile cast nephropathy. Pigmented casts are seen (arrows) (H&E, ×200).

Fig. 3

Tubular cast (arrow) positive on Hall’s stain (×400).

Fig. 4

Severe fibrointimal thickening of two arteries (arrows) as a histological sign of the severe nephroangiosclerosis (Jones methenamine silver stain, ×200).