Bilirubin Removal with Therapeutic Plasma Exchange or Molecular Adsorbent Recirculating System as Treatment for Cholemic Nephropathy in Patients with Cirrhosis and Acute-on-Chronic Liver Failure: A Case Series.

Introduction: Cholemic nephropathy is an overlooked cause of acute kidney injury (AKI) in patients with cirrhosis and high bilirubin plasma levels (usually above 20 mg/dL), due to bilirubin and bile acid deposition in the kidneys. Those deposits have been hypothesized to cause tubular injury. It has no standardized diagnostic criteria or therapeutic strategies.

Method: We present a series of 15 patients with cirrhosis, acute-on-chronic liver failure (ACLF), and severe cholemic AKI, diagnosed by microscopic urinary cast visualization after excluding and treating other causes of AKI. Bilirubin plasma removal was performed with Molecular Adsorbent Recirculating System (MARS®, n = 3) or therapeutic plasma exchange (TPE, n = 12) to treat and prevent further kidney deterioration.

Results: Kidney function improved in most of the patients; 5 patients also required transient hemodialysis, with only 1 patient evolving to end-stage chronic kidney disease needing liver-kidney transplant. Five patients underwent extended TPE sessions as a bridge to liver transplantation. Survival at 30 days and 1 year was 80% and 73%, respectively, with 10 patients undergoing transplantation along this year.

Conclusion: In this highly selected cohort of patients with cirrhosis, ACLF, and severe cholemic AKI, extracorporeal plasma removal techniques seem to improve kidney function and overall prognosis. Larger prospective and controlled studies are required to better understand this condition.

Introduction: Cholemic nephropathy is an overlooked cause of acute kidney injury (AKI) in patients with cirrhosis and high bilirubin plasma levels (usually above 20 mg/dL), due to bilirubin and bile acid deposition in the kidneys. Those deposits have been hypothesized to cause tubular injury. It has no standardized diagnostic criteria or therapeutic strategies.

Method: We present a series of 15 patients with cirrhosis, acute-on-chronic liver failure (ACLF), and severe cholemic AKI, diagnosed by microscopic urinary cast visualization after excluding and treating other causes of AKI. Bilirubin plasma removal was performed with Molecular Adsorbent Recirculating System (MARS®, n = 3) or therapeutic plasma exchange (TPE, n = 12) to treat and prevent further kidney deterioration.

Results: Kidney function improved in most of the patients; 5 patients also required transient hemodialysis, with only 1 patient evolving to end-stage chronic kidney disease needing liver-kidney transplant. Five patients underwent extended TPE sessions as a bridge to liver transplantation. Survival at 30 days and 1 year was 80% and 73%, respectively, with 10 patients undergoing transplantation along this year.

Conclusion: In this highly selected cohort of patients with cirrhosis, ACLF, and severe cholemic AKI, extracorporeal plasma removal techniques seem to improve kidney function and overall prognosis. Larger prospective and controlled studies are required to better understand this condition.