Although antineutrophil cytoplasmic antibodies (ANCA) were first associated with the primary vasculitides, it is now clear that 15-20% of patients with lupus have detectable ANCA. In this short review we confirm that the major link is with perinuclear ANCA (pANCA) but not cytoplasmic ANCA (cANCA). ANCA to myeloperoxidase are associated with drug-induced lupus. There may be a link between pANCA levels and disease activity in some patients although the links to specific organ involvement are not proven. ANCA in lupus must be interpreted cautiously with particular attention paid to laboratory technique, the size, age and genetic background of the populations studied.