li-11-9 AUTOIMMUNE CHOLANGITIS By Dr. E. Orfei
AMA (AntiMitochondrialAntibody) negative intrahepatic cholangiopathy
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).
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.
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.
Prednisone treatment induces rapid clinical an biochemical remission.