Local esperience on frequency and significance of immunological overlap in the field of primary biliary cirrhosis (PBC)

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Caterina Defendenti *
Simona Bollani
Gabriella Munegato
Simone Saibeni
Maria Sofia
Savino Bruno
Mariella Saudelli
Maurizio Picca
(*) Corresponding Author:
Caterina Defendenti | caterina.defendenti@fbf.milano.it

Abstract

The aim of this study was to investigate the frequency and quality of immunological overlap in serum AMA M2 positives. During the period January 2004 - December 2006, out of 3000 sera evaluated, 40 were AMA M2 positive. Six of these (15%) were without immunological overlap; they had clinical, histological and biochemical features of classical PBC with a normal length of life and ursodeoxycholic acid responding. In other 16 patients (40%), there was an association with antibody against antigens as SLA/LP, gp210 and Sp100, that causes the overlap syndrome AIH/PBC with various level of seriousness.The association with anti SLA/LP or with high level of autoantibody anti-Sp100 (index ELISA>10) waspresent in the patients investigated, as AIH type I°. Patients positive for anti gp210 with low anti-Sp100 index had instead a weak overlap AIH/PBC with slow evolving aspects. Nobody was positive for anti-LKM1, anti-LC, PML, or LBR.The remaining 45% of sera were positive for ANA, ENA,ANCA, with different clinical features. The antibody anti-SSARo52 and anti-centromere were frequent (17%) and modified the liver disease evolution.The most serious clinical case was a double overlap anti-centromere/SSARo52 with biochemical and histological AIH symptoms. Other ENA patterns did worsen the prognosis of PBC but showed or were associated with systemic diseases, often after a long asymptomatic period.The ANA IFI positive with a speckled pattern ENA negative, in other 7 patients (17%) worsened the long term prognosis because they expressed an overlap AIH/PBC with slowly evolving aspects.The most serious and intense clinical picture, with transplant perspective, was the association of the AMA M2 with anti Sp100 (index > 10) and pANNA. Four case were male (10%) but females were prevalent.

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