The hepatitic/cholestatic "overlap" syndrome: an Italian experience.
BACKGROUND
Patients with hepatitic and cholestatic autoimmune liver disease ("overlap syndrome") represent a diagnostic and therapeutic challenge.


OBJECTIVE
To evaluate the prevalence of the "hepatitic/cholestatic overlap" in a large series of consecutive patients with cholestatic autoimmune liver disease.


METHODS
We re-evaluated the diagnosis of 235 patients with autoimmune liver disease, including 70 with type 1 autoimmune hepatitis (AIH), 142 with primary biliary cirrhosis (PBC), and 23 with primary sclerosing cholangitis (PSC), using the revised International Autoimmune Hepatitis Group (IAIHG) scoring system. Anti-mitochondrial, anti-nuclear, anti-smooth muscle, anti-liver kidney microsomal type 1, anti-liver cytosol type 1, perinuclear anti-neutrophil nuclear and anti-soluble liver antigen antibodies were evaluated in each patient.


RESULTS
Ten patients (3 with a previous diagnosis of PBC and 7 of PSC) scored as "probable" or "definite" AIH. These patients did not have a specific autoantibody profile.


CONCLUSIONS
Among patients with PBC, the occurrence of a PBC/AIH overlapping syndrome is rare (2.1%), whereas among patients with PSC an overlap between PSC and AIH is frequent (30.4%). Whether patients with the hepatitic/cholestatic overlap syndrome would benefit from a combination therapy with immunosuppression and ursodeoxycholic acid remains to be established.
Muratori
Luigi
L
Department of Internal Medicine, Cardioangiology, Hepatology, Alma Mater Studiorum, University of Bologna, Policlinico Sant'Orsola-Malpighi, via Massarni, 9, 40138, Bologna Italy. muratori@med.unibo.it
Cassani
Fabio
F
Pappas
Georgios
G
Guidi
Marcello
M
Mele
Loredana
L
Lorenza
Veronesi
V
Lenzi
Marco
M
Bianchi
Francesco B
FB
Muratori
Paolo
P
D001323:Autoantibodies / Q000276:immunology*; D005260:Female; D019693:Hepatitis, Autoimmune / Q000276:immunology*; D006801:Humans; D007558:Italy; D008105:Liver Cirrhosis, Biliary / Q000276:immunology*; D008297:Male
D001323:Autoantibodies