Benign recurrent intrahepatic cholestasis: review and long-term follow-up of five cases.
OBJECTIVE
Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by intermittent episodes of cholestatic jaundice. For the patients, the disease is a physical and psychological challenge. There is no curable treatment, but symptomatic relief is described following treatment with rifampicin or plasmapheresis.


METHODS
Five patients suffering from BRIC followed up for 17 years by one consultant are described. Two patients were treated with rifampicin and plasmapheresis, two with rifampicin alone, and one with plasmapheresis.


RESULTS
The treatments showed symptomatic relief, effect on biochemical parameters, and earlier clinical remission compared with no treatment or treatment with other substances like cholestyramine, antihistamines, and ursodeoxycholic acid.


CONCLUSIONS
Both rifampicin and plasmapheresis represent important therapeutic options of acute cholestatic attacks in patients with BRIC. As a noninvasive treatment, rifampicin may be the first choice.
Department of Medicine, Division of Gastroenterology, Haukeland University Hospital and Institute of Medicine, University of Bergen, Bergen, Norway. geir.folvik@helse-bergen.no
Geir
G
Folvik
Olset
O
Hilde
Gilja Odd
GO
Helge
D000328:Adult; D000904:Antibiotics, Antitubercular / Q000627:therapeutic use*; D001663:Bilirubin / Q000097:blood; D002780:Cholestasis, Intrahepatic / Q000235:genetics / Q000628:therapy*; D003131:Combined Modality Therapy; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D012008:Recurrence; D012293:Rifampin / Q000627:therapeutic use*; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000008:administration & dosage; D055815:Young Adult
D000904:Antibiotics, Antitubercular; D014580:Ursodeoxycholic Acid; D001663:Bilirubin; D012293:Rifampin