Ursodeoxycholic acid for treatment of cholestasis in patients with hepatic amyloidosis.
BACKGROUND
Amyloidosis represents a group of different diseases characterized by extracellular accumulation of pathologic fibrillar proteins in various tissues and organs. Severe amyloid deposition in the liver parenchyma has extrahepatic involvement predominantly in the kidney or heart. We evaluated the effect of ursodeoxycholic acid, in four patients with severe hepatic amyloidosis of different etiologies, who presented with increased alkaline phosphatase and gamma-glutamyl transferase.


METHODS
The study included four patients who presented with amyloidosis-associated intrahepatic cholestasis. Three of them had renal amyloidosis which developed 1-3 years before cholestasis occurred, the remaining one having intrahepatic cholestasis as the primary sign of the disease. Amyloidosis was identified from liver biopsies in all patients by its specific binding to Congo red and green birefringence in polarized light. The biochemical nature and the class of amyloid deposits were identified immunohistochemically. In addition to their regular treatment, the patients received 750 mg ursodeoxycholic acid per day. After 2-4 weeks all patients had a significant decrease of serum alkaline phosphatase and gamma-glutamyl transferase, and their general status significantly improved.


CONCLUSIONS
Treatment with ursodeoxycholic acid may be beneficial in patients with hepatic amyloidosis, and do extend indications for the use of ursodeoxycholic acid in amyloidotic cholestatic liver disease.
Faust
Dominik
D
Asklepios Hospital, Department of Medicine, Langen, Germany. innere2.longen@asklepios.com
Akoglu
Bora
B
Ristic
Gordana
G
Milovic
Vladan
V
D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000150:complications*; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D002779:Cholestasis / Q000188:drug therapy* / Q000209:etiology; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000150:complications*; D008297:Male; D008875:Middle Aged; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid