Bile cast nephropathy: A case report and review of the literature

Abstract

Bile cast nephropathy is a condition of renal dysfunction in the setting of hyperbilirubinemia. There are very few cases of this condition reported in the last decade and a lack of established treatment guidelines. While the exact etiology remains unknown, bile cast nephropathy is presumed to be secondary to multiple concurrent insults to the kidney including direct toxicity from bile acids, obstructive physiology from bile casts, and systemic hypoperfusion from vasodilation. Therapy directed at bilirubin reduction may improve renal function, but will likely need dialysis or plasmapheresis as well. We report our case of bile cast nephropathy and the therapeutic measures undertaken in a middle-aged male with chronic renal insufficiency that developed hyperbilirubinemia and drug-induced liver injury secondary to antibiotic use. He developed acute renal injury in the setting of rising bilirubin. He subsequently had a progressive decline in renal and hepatic function, requiring dialysis and plasmapheresis with some improvement, ultimately requiring transplantation.

Cholemic Nephropathy Causes Acute Kidney Injury and Is Accompanied by Loss of Aquaporin 2 in Collecting Ducts

“…In this context, an association of cholestasis with the impairment of renal function has already described at the beginning of the last century . Since then, most of the data on this disease entity, which is known as cholemic nephrosis, bile cast nephropathy or CN, has been derived from case reports or autopsy studies . However, none of the studies systematically investigated the frequency of CN in a living cohort.…”

“…Even though CN was first described in the early 20th century, this disease entity has been neglected until recently. Moreover, most of the data on patients with CN were derived from case reports or autopsy studies . The diagnostic work‐up of impaired renal function in the context of liver cirrhosis usually includes noninvasive diagnosis and does not include kidney biopsy owing to coagulopathy with increased risk of bleeding .…”

Role of inflammation and inflammasome activation in human bile cast nephropathy

“…Bile cast nephropathy is a largely forgotten diagnosis with a poorly understood pathophysiology . Bile casts can play an important role in the pathogenesis of kidney dysfunction both in patients with and without chronic liver impairment .…”

“…High serum bilirubin, or hyperbilirubinaemia, results from disorders that occur in any of the multiple steps of conjugation and excretion . In individuals with profound hyperbilirubinaemia, increased bilirubin filtration may lead to the formation of bile casts . Both bilirubin toxicity and tubular obstruction by bile casts have been implicated in the pathogenesis of acute kidney injury (AKI), commonly known as bile cast nephropathy (BCN) .…”

“…In individuals with profound hyperbilirubinaemia, increased bilirubin filtration may lead to the formation of bile casts . Both bilirubin toxicity and tubular obstruction by bile casts have been implicated in the pathogenesis of acute kidney injury (AKI), commonly known as bile cast nephropathy (BCN) . BCN has been described as a spectrum of renal injuries from proximal tubulopathy to intratubular cast formation in patients with severe liver dysfunction .…”