Perinuclear Antineutrophil Cytoplasmic Antibodies pANCAs and Antibodies to Asialoglycoprotein Receptor antiASGPRs

Perinuclear anti-neutrophil cytoplasmic antibodies occur in 65-96% of patients with AIH type 1 but are usually absent in patients with AIH type 2 [24, 25]. They are mainly of the IgG1 isotype, which distinguishes them from the pANCAs in primary sclerosing cholangitis [24]. Typically, these antibodies are present in high titers in AIH type 1. Actin has been proposed as target antigen; however, recent studies suggested reactivity against granulocyte-specific antigens in the nuclear lamina [26, 27]. These antibodies are of diagnostic value for those patients with AIH type 1 who have tested negative for ANA/SMA.

Anti-ASGPR is present in all forms of disease [28-30]. Seropositivity is associated with laboratory and histological indices of disease activity, and anti-ASGPRs identify patients who commonly will have a relapse after corticosteroid withdrawal [31, 32]. However, anti-ASGPRs are also found in other liver diseases including alcoholic liver disease, chronic hepatitis B and C, and primary biliary cirrhosis. The development of a commercial assay for application of anti-ASGPRs has been difficult, and their potential as diagnostic and prognostic markers has not yet been realized.

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