Abstract
BACKGROUND: Wilson's disease (WD) is a rare inherited disorder of copper metabolism. Treatment consists of chelating agents, but side effects are common. We describe a patient who developed colitis during trientine treatment leading to decompensation of liver cirrhosis. CASE SUMMARY: A healthy 51-year-old woman was diagnosed with liver cirrhosis due to decompensation with ascites. Etiologic evaluation raised suspicion of hereditary hemochromatosis because of compound heterozygosity HFE p.C282Y/p.H63D, and phlebotomy was started. Re-evaluation showed low ceruloplasmin, increased urinary copper excretion and the presence of Kayser-Fleischer rings. WD was confirmed by genetic analysis. Because of decompensated cirrhosis, she was referred for liver transplant evaluation. Simultaneously, treatment with trientine was initiated. Liver function initially stabilized, and the patient was not accepted for a liver transplant. Shortly after this, she developed severe hemorrhagic colitis, most probably a side effect of trientine. During that episode, she decompensated with hepatic encephalopathy. Because of a second decompensating event, she was accepted for liver transplantation, and an uneventful transplantation was carried out after clinical improvement of colitis. CONCLUSION: Despite WD being a rare disorder, it is important to consider because it can present with a plethora of symptoms from childhood to an elderly age. Colitis should be recognized as a serious adverse drug reaction to trientine treatment that can result in decompensated liver disease.