Another open-label multicenter international phase IIa treatment trial (clinicaltrials.gov#: NCT02128269) is evaluating the safety and tolerability of an intravenous C5a inhibitor in persistently aPL-positive patients with at least one of the following non-criteria manifestations of APS: aPL-nephropathy, skin ulcers, and/or thrombocytopenia. This evidence concerns the gene C5 and autoimmune polyendocrinopathy.