[Presence of SLA/LP autoantibodies in patients with primary biliary cirrhosis as a marker for secondary autoimmune hepatitis (overlap syndrome)].
OBJECTIVE
The aim of this study was to evaluate whether the presence of SLA/LP-autoantibodies in PBC-patients gives evidence for a secondary AIH, also called AIH/PBC-overlap-syndrome.


METHODS
Out of 233 consecutive patients with PBC who had been followed between October 1980 and April 2000, we evaluated the data of anti-SLA/LP-positive patients and compared them to patients with an anti-SLA/LP-negative AIH/PBC overlap syndrome as well as to patients with a classical course of AIH and PBC.


RESULTS
In total we could identify nine PBC patients with anti-SLA/LP antibodies (six women/three men) or 3.9% of the study population, Anti-SLA/LP-positive PBC patients were slightly younger at diagnosis in comparison to anti-SLA/LP-negative PBC-patients (49.9 vs. 53.2 years). Transaminases and gamma-globulins were significantly higher in anti-SLA/LP-positive PBC-patients in comparison to anti-SLA/LP-negative PBC-patients (mean: 235 vs. 55 IU/l and 27.6 vs. 19.5 g/l). Anti-SLA/LP-positive patients significantly more frequently had an HLA-type that is characteristic for AIH (B8; DR3; DR4). Immunosuppressive therapy reduced inflammatory activity and cholestasis significantly. Relapses were frequent after reduction or discontinuation of immunosuppressive therapy.


CONCLUSIONS
The presence of SLA/LP autoantibodies in PBC patients has a high specificity for a secondary AIH (AIH/PBC overlap syndrome). These patients have a good response to immunosuppressive therapy. The autoantibody profile and immunogenetics may help in future to identify PBC patients that benefit most from immunosuppressive therapy.
Kanzler
S
S
I. Medizinische Klinik und Poliklinik, Johannes Gutenberg Universität, Mainz.
Bozkurt
S
S
Herkel
J
J
Galle
P R
PR
Dienes
H P
HP
Lohse
A W
AW
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D001706:Biopsy; D015153:Blotting, Western; D004796:Clinical Enzyme Tests; D003627:Data Interpretation, Statistical; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005455:Fluorescent Antibody Technique; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000276:immunology* / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D000637:Transaminases / Q000097:blood; D005719:gamma-Globulins / Q000032:analysis
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D005719:gamma-Globulins; D000637:Transaminases