Overlap syndrome: autoimmune hepatitis with primary biliary cirrhosis.
"Overlap syndrome" is used to describe variant forms of autoimmune hepatitis (AIH) which present with characteristics of AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC. AIH-PBC is the most common form of overlap syndrome, affecting almost 10% of adults with AIH or PBC. Transitions from PBC to AIH-PBC overlap syndrome have also been reported. Overlap syndromes show a progressive course without treatment, and therapy is empiric. Ursodeoxycholic acid is usually combined with immunosuppressive therapy but end-stage disease requires liver transplantation. We report a case of AIH/PBC overlap with relevant discussion of literature.
Department of Digestive Health and Diseases, (Government Peripheral Hospital), Kilpauk Medical College Hospital, Chennai 600 102.
S
S
Arulprakash
Anand D
AD
Sasi
Murali R
MR
Bala
T
T
Pugazhendhi
S Jeevan
SJ
Kumar
D000328:Adult; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001706:Biopsy; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D005260:Female; D019693:Hepatitis, Autoimmune / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D013577:Syndrome; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D001323:Autoantibodies; D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid