The serological profile of the autoimmune hepatitis/primary biliary cirrhosis overlap syndrome.
OBJECTIVE
During the last decade patients with concomitant clinical, biochemical, immunoserological, and histological features of both autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) were sporadically described, but definite diagnostic criteria and specific serological markers to support the diagnosis of AIH/PBC overlap syndrome (AIH/PBC OS) are still lacking.


METHODS
Clinical, biochemical, and histological features, autoantibody profile, and treatment response of 15 patients with coexistent hepatitic and cholestatic liver damage, all fulfilling strict diagnostic criteria for both AIH and PBC, were compared with those of 120 patients with pure PBC and 120 patients with pure AIH.


RESULTS
At diagnosis, the AIH/PBC OS patients' median age was 51 years, similar to that of the PBC patients (52 years, P=NS), but significantly higher than that of the AIH patients (40 years, P=0.04). Anti-dsDNA antibodies were detected in 60% of AIH/PBC OS patients, but only in 4% of PBC patients and 26% of AIH patients (P<0.0001 and 0.01, respectively). Double positivity for antimitochondrial antibodies (AMA) and anti-dsDNA was present in 47% of those with AIH/PBC OS, but only in 2% of the pathological controls (P<0.0001; specificity: 98; 95% confidence interval (CI): 97-99.2; positive likelihood ratio: 28; 95% CI: 9.8-79.4). Combined therapy (ursodeoxycholic acid (UDCA) plus steroids) achieved biochemical response in 77% of AIH/PBC OS patients.


CONCLUSIONS
Concomitant AMA/anti-dsDNA seropositivity can be considered the serological profile of AIH/PBC OS. The combination of UDCA and steroids is effective in achieving persistent biochemical amelioration in most AIH/PBC OS patients.
Muratori
Paolo
P
Department of Clinical Medicine, Alma Mater Studiorum, University of Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti, 9, Bologna, Italy. paolo.muratori3@unibo.it
Granito
Alessandro
A
Pappas
Georgios
G
Pendino
Gaspare M
GM
Quarneti
Chiara
C
Cicola
Ronny
R
Menichella
Rita
R
Ferri
Silvia
S
Cassani
Fabio
F
Bianchi
Francesco B
FB
Lenzi
Marco
M
Muratori
Luigi
L
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000276:immunology*; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000276:immunology; D011379:Prognosis; D011860:Radiography, Abdominal; D012189:Retrospective Studies; D013577:Syndrome; D055815:Young Adult
D000974:Antibodies, Antinuclear