Characterization of overlap syndrome between primary biliary cirrhosis and autoimmune hepatitis according to antimitochondrial antibodies status.
OBJECTIVE
Codification of variant forms between Primary Biliary Cirrhosis (PBC) and Autoimmune Hepatitis (AIH) has not been definitively standardized. The aim of this study was to compare among 102 consecutive patients, 2 subsets of overlap syndrome (OS, N=21) with and without antimitochondrial antibody (AMA) to two groups of patients with typical PBC (N=43) or AIH (N=38).


METHODS
OS was defined by the presence in the same patient of at least 2 of 3 accepted criteria of PBC and AIH. Twelve patients with OS were AMA negative and 9 were AMA positive.


RESULTS
A lower level of alanine transaminase (139+/-48 vs 269+/-154 IU/L, P<0.05) and a trend towards a higher level of alkaline phosphatase or gamma-glutamyl transpeptidase was observed in OS without AMA than in OS with AMA (693+/-200 vs 544+/-124 IU/L; 370+/-66 vs 241+/-77 IU/L, respectively). All AMA-negative patients with OS had antinuclear and/or anti-smooth muscle antibodies. OS without AMA differed from those with AMA in that they had more severe bile duct damage including destructive cholangitis (P<0.05), ductopenia (P<0.05), ductular hyperplasia (P<0.05) and a higher METAVIR fibrosis score (2.5+/-0.3 vs 1.3+/-0.3, P<0.05). The response to therapy was not different between PBC, AIH and OS.


CONCLUSIONS
According to the presence of AMA, 2 homogeneous subgroups of patients with overlap syndrome between PBC and AIH may be identified. AMA status affects clinical presentation and liver disease severity of OS.
Alric
Laurent
L
Service de Médecine Interne, Fédération Digestive, Pavillon Dieulafoy, CHU Purpan, Toulouse. ALRIC.L@chu-toulouse.fr
Thebault
Sophie
S
Selves
Janik
J
Peron
Jean-Marie
JM
Mejdoubi
Sanae
S
Fortenfant
Françoise
F
Vinel
Jean-Pierre
JP
D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D000469:Alkaline Phosphatase / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000201:enzymology / Q000276:immunology*; D006801:Humans; D008099:Liver / Q000276:immunology; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000188:drug therapy / Q000201:enzymology / Q000276:immunology*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000276:immunology*; D013577:Syndrome; D016896:Treatment Outcome; D005723:gamma-Glutamyltransferase / Q000097:blood
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D005723:gamma-Glutamyltransferase; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase