Possible role of endogenous retinoid (Vitamin A) toxicity in the pathophysiology of primary biliary cirrhosis.
Primary biliary cirrhosis (PBC) is a chronic, cholestatic disease of unknown etiology commonly affecting women. It is characterized by progressive destruction of the small intrahepatic bile ducts and portal inflammation, leading to fibrosis and cirrhosis. The major signs and symptoms of PBC, which include pruritus, lethargy, the sicca syndrome, and osteoporosis, closely resemble the manifestations of hypervitaminosis A. Based on a review of the literature and other observations connecting PBC with retinoid metabolism (vitamin A and its derivatives), the hypothesis is proposed that exposure to excess endogenous retinoids contributes to the pathogenesis of PBC and may be to the cause of some of the signs and symptoms associated with the disease.
Erickson
J M
JM
Department of Internal Medicine, Iowa Methodist Medical Center, 1215 Pleasant St., Suite 300, Des Moines, IA 50309, USA.
Mawson
A R
AR
D000328:Adult; D005260:Female; D006801:Humans; D006986:Hypervitaminosis A / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D008099:Liver / Q000378:metabolism* / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D008875:Middle Aged; D008954:Models, Biological; D014801:Vitamin A / Q000378:metabolism
D014801:Vitamin A