Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis: a retrospective study of 115 cases of autoimmune liver disease.
OBJECTIVE
The aim of this retrospective study was to compare clinical, biological, and histological features and treatment response in 115 patients with overlap syndrome (OS), autoimmune hepatitis (AIH) or primary biliary cirrhosis (PBC).


METHODS
Consecutive patients with AIH, PBC or OS followed between 1984 and 2005 in five different centers were included. All data were re-evaluated using current diagnostic criteria of each disease.


RESULTS
Fifteen patients had OS (13 females), 48 AIH (40 females) and 52 PBC (49 females). Patients with OS were significantly younger than patients with PBC (median age: 44 vs 59 years). Jaundice (20%) and pruritus (20%) were the main initial symptoms in OS. Patients with OS had serum transaminase and gammaglobulin levels significantly higher than patients with PBC; serum alkaline phosphatase, gamma-glutamyl-transpeptidase and IgM levels were significantly higher in OS than in patients with AIH. Histological analysis showed moderate or severe piecemeal necrosis in 86% and destructive cholangitis in 93% in OS group. Among 11 patients with OS treated with ursodeoxycholic acid (UDCA) or immunosuppressors alone, only 6 had a complete biochemical response. In contrast, all patients with OS receiving combined therapy, as first or second line, responded, 5 patients to the combination corticosteroids-azathioprine-UDCA and 2 to the combination cyclosporine-UDCA.


CONCLUSIONS
OS is not rare and accounts for 13.9% of patients with autoimmune liver disease in our series. Combination of immunosuppressors and UDCA appears the most efficient treatment in these patients.
Heurgué
Alexandra
A
Service d'Hépato-Gastroentérologie; CHU Reims.
Vitry
Fabien
F
Diebold
Marie-Danièle
MD
Yaziji
Nahla
N
Bernard-Chabert
Brigitte
B
Pennaforte
Jean-Loup
JL
Picot
Rémi
R
Louvet
Hervé
H
Frémond
Luc
L
Geoffroy
Patrick
P
Schmit
Jean-Luc
JL
Cadiot
Guillaume
G
Thiéfin
Gérard
G
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000188:drug therapy; D004359:Drug Therapy, Combination; D005260:Female; D005602:France; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000473:pathology*; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy*; D008111:Liver Function Tests; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid