An autopsy case of a patient on maintenance hemodialysis with continuous idiopathic cholesterol crystal embolism for 7 years

Abstract

Background: CCE is a systemic disease with poor prognosis with no established treatment. Approximately 23–32% of CCE cases progress to end-stage renal failure, and the 1-year mortality rate of CCE with organ failure is 60–90%. The dialysis method for the patients with CCE is still controversial. Case report: The patient is 73 years old male who was diagnosed with idiopathic CCE. He had survived 7 years though he had been on maintenance HD. We used nafamostat for HD every time. He took prednisolone and statin. He died due to rupture of AAA and we autopsied him. CCs developed in five organs, including the right lung CCE was assumed to be continuously present since the diagnosis. Discussion and conclusion: CCE was continuous until death, and CCs in the right lung were possibly due to HD. HD through AV shunt could worsen CCE, and HD should be recognized as the aggravating factor. The use of nafamostat while undergoing HD as well as use of steroids and statins until death may have prevented fatal events and contributed to the patient's long survival.