Arterial occlusion with subsequent amputation of extremities is a rare manifestation of systemic lupus erythematosus (SLE). It may be caused by local arteritis and/or thrombosis. We describe the clinical and laboratory manifestations and treatment administered to six SLE patients who developed peripheral arterial necrosis necessitating amputation of extremities secondary to the arterial occlusion. All patients were female, with ages ranging from 16 to 65 years. Arterial occlusion took place in the initial months of disease (median: 7 months). Only one of five patients tested for antiphospholipid antibodies had these antibodies who also had vasculitis and thrombosis in a histopathological study. Most patients presented a very benign outcome after the amputation of extremities and stayed in remission for several years. The satisfactory outcome of most patients after the vascular phenomenon allows us to consider the possibility that such a complication could be, for unknown reasons, a marker for good prognosis in SLE or, alternatively, that the aggressive therapy administrated for patients with this complication at the beginning of the disease could recover the balance of the immune system, avoiding future relapses.