SP102
HEPATOTOXICITY ASSOCIATED WITH ECULIZUMAB TREATMENT Free

INTRODUCTION AND AIMS: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threating disease characterized by systemic thrombotic microangiopathy (TMA) which is associated with uncontrolled activation of C5 due to dysregulation of alternative complement pathway. Eculizumab is monoclonal C5 anti-body, approved for aHUS treatment which is reported as efficient and safe in clinical trials. Although transient transaminase enzyme elevation was reported mostly in patients prior to treatment, hepatotoxicity in association with Eculizumab has not been reported in adults previously.

METHODS: Herein we presented an adult case of aHUS in whom hepatotoxicity was observed following Eculizumab treatment.

RESULTS: A 39 year old male was referred to our center with acute renal failure, hypertension, blurred vision, and thrombocytopenia. After exclusion for possible causes of TMA, diagnosis of aHUS was considered and plasma exchange (PE) was initiated in the first 24 hours. Eculizumab was administered because of PE dependence and persistent renal failure after 19 PE sessions. Although TMA improved without recovery of renal functions under Eculizumab treatment, we had to withdraw Eculizumab treatment because of drug induced liver injury with elevated transaminases. After the 4^th dose of Eculizumab marked elevation in enzyme levels (ALT 471 IU/L, x8 UNL (upper normal limit), ALP 516 IU/L, x4 UNL and GGT 1001 IU/L, x15 UNL) were observed and we had to stop Eculizumab because of fulminant hepatitis risk. Other causes for elevated transaminases were excluded and liver enzymes were normalized within 20 days with recovery after cessation. He continues to our outpatient clinic on anti-hypertensive treatment and free of TMA event with no abnormalities in liver enzyme levels.

CONCLUSIONS: Drug induced liver injury should be kept in mind as an important adverse event related with Eculizumab which is not reported previously in adults. We suggest monitoring transaminase levels in patients receiving Eculizumab and further studies are required to evaluate hepatotoxicity risk. To our knowledge this is the first case report of severe hepatotoxicity associated with Eculizumab treatment.