AUTOANTIBODY PROFILE OF PRIMARY SCLEROSING CHOLANGITIS

Primary sclerosing cholangitis (PSC) is a chronic progressive liver di sease of unknown etiology. It has been suggested that genetic and immu nological factors are important in its pathogenesis. The present study examined the prevalence of 23 different autoantibodies in 25 PSC sera , by ELISA, in order to better define the autoimmune profile of PSC. T he results indicate that 88% of PSC patients produced at least 1 autoa ntibody, and 36% had reactivity to multiple autoantibodies. Moreover, 35% of the PSC patients produced antiendothelial-cell antibodies (AECA ) and 75% of the sera contained perinuclear antineutrophil cytoplasmic antibodies (pANCA), detected by indirect immunofluorescence. The prom inent ANCA autoantibody was anti-cathepsin-G, demonstrated in 35% of t he patients, The multiplicity of the autoantibody profile, revealed in the present study, points to the autoimmune characteristics of PSC. I n addition, the association of ANCA and of AECA in PSC may suggest a p athogenic role for these antibodies in PSC.