Association of ANCA isotype and affinity with disease expression

The association of ANCA isotype and affinity with disease expression is reviewed and new data on IgA ANCA levels and affinity in relation to disease activity are presented. Differences in the IgG ANCA subclass distribution exist between active disease and remission, with high affinity IgG3 ANCA present at the onset of disease. IgM ANCA, with or without associated IgG ANCA, correlates with pulmonary haemorrhage and disappears at remission. IgA ANCA are mainly found in patients presenting with Henoch-Schönlein purpura (HSP), and are directed against a distinct autoantigen of approximately 50 kD. The level and affinity of IgA ANCA also correlate with disease activity in HSP. Isotype as well as antibody specificity appear to be major determinants of disease expression.