<p><b>OBJECTIVE</b>In order to provide a reliable basis for the
diagnosis and
treatment of
autoimmune hepatitis (
AIH) and its overlap
syndrome, we investigated the clinical, immunological characteristics of and the
therapeutic methods for
AIH and
AIH-
primary biliary cirrhosis (PBC) overlap
syndrome.</p><p><b>
METHODS</b>One hundred seven
patients (77 with
AIH and 30 with
AIH-PBC overlap
syndrome) were enrolled in the study. Their clinical manifestations,
serum liver function tests (LFTs) findings,
serum immunoglobulins,
liver histopathological changes and their responsiveness to the
therapies were investigated.</p><p><b>RESULTS</b>The
age distribution of
AIH patients showed a single peak during their fifties and their main clinical manifestations were malaise, abdominal distension,
anorexia and
jaundice.
Serum gamma globulin and
IgG were significantly higher than their normal levels. 74% of the
patients were positive for anti-nuclear antibody (ANA), 32% of the
patients were positive for anti-
smooth muscle antibody (AMA), and over 50% of the
patients suffered from concurrent extrahepatic
autoimmune diseases. The main histological changes in the
liver biopsies were interface
hepatitis (65%), lobular
hepatitis and
rosette formation of
liver cells. Bridging
necrosis was observed in severe
AIH cases. In the
AIH-PBC overlap
syndrome patients, the levels of
serum ALT, AST, GGT, ALP and
incidences of ANA and AMA/AMA-M2 were all significantly higher than those of the
AIH group. After treating
AIH patients with
prednisolone and
azathioprine (Aza), complete response was seen in 42 cases (70%), sustained response was seen in 26 cases (43%). Sixteen cases had
relapses after the withdrawal of the
treatment or
prednisolone dosage was reduced lower than 10 mg/d. The cases having normal
serum ALT, AST,
gamma-globulin and
IgG levels
after treatment were still responding to the reduced
prednisolone dosage of 5-10 mg/d without
azathioprine added. After combination with
ursodeoxycholic acid (UDCA)
treatment, the
liver function tests (AST, ALT, TBil) of
AIH-PBC overlap
syndrome patients also significantly improved compared to those before the
treatment (P<0.01).</p><p><b>CONCLUSION</b>
AIH and
AIH-PBC overlap
syndrome are not rare in our clinics. Their
diagnoses should be based on the clinical presentations, biochemical and immunological indices and
liver histological changes. In
AIH cases, once their AST, ALT,
gamma-globulin and
IgG levels return to normal, the
prednisolone dosage can be maintained at 5-10 mg/d and Aza can even be withdrawn. Good improvement for
patients with
AIH-PBC overlap
syndrome can be obtained with UDCA and
immunosuppression treatment.</p>