Anti-neutrophil antibodies in primary sclerosing cholangitis

Abstract

Anti-neutrophil specific antibodies are detected in up to 88% of patients with PSC. The labelling pattern of neutrophils produced by these antibodies when examined by indirect immunofluorescence microscopy is distinct from that produced by anti-neutrophil cytoplasmic antibodies (ANCA) in vasculitic diseases. The antigen(s) recognized by anti-neutrophil antibodies in PSC is not yet known but appears to be localized to the periphery of the nucleus. Accordingly, the term peripheral anti-neutrophil nuclear antibodies (p-ANNA) is more appropriate than the frequently used p-ANCA. As the titre of p-ANNA in PSC does not correlate with disease-specific clinical parameters, they are not useful markers for the management of patients. However, the high prevalence of p-ANNA in PSC makes them a reasonable diagnostic marker if used in conjunction with other standard diagnostic tests. The role of anti-neutrophil antibodies in the pathogenesis of PSC, if any, remains to be established.