li-11-9 AUTOIMMUNE CHOLANGITIS By Dr. E. Orfei
DEFINITION
AMA (AntiMitochondrialAntibody) negative intrahepatic cholangiopathy
HISTORY
First noticed in 15 patients. by Brunner G & Klinge O in 1987 (Dtsch Med. Wochschr.112:1454,1987) .
Described in one patient by Corrangher JG et al.( Semin. Liver diseases 1:252,1991).
Reported in 3 patients by Micheletti P et al.(Gut,35:260,1994).
BIOCHEMISTRY
AMA negative
ANA positive.
Other laboratory tests similar values as in Primary biliary Cirrhosis.
The syndrome is different from Primary Biliary cirrhosis because is AMA negative. It is Also different from Idiopatic Adulthood Ductopenia Because it is AMA and ANA negative.
PATHOLOGY
Periductal edema and inflammation with plasma cells and some lymphocytes.
portal and septal bile duct damage with intraepithelial lymphocytes.
Portal and periportal inflammation with piece-meal necrosis
Intra-acinar (lobular) hepatitis.
TREATMENT
Prednisone treatment induces rapid clinical an biochemical remission.