Management of Non-criteria Manifestations in Antiphospholipid Syndrome

To review the available evidence on the management of a variety of non-criteria manifestations in antiphospholipid syndrome (APS), including valvular disease, alveolar hemorrhage, thrombocytopenia, hemolytic anemia, APS nephropathy, skin ulcers, livedo reticularis, cognitive dysfunction, and epilepsy.

Current treatment relies on low-level evidence and mainly on expert consensus due to the rarity and the heterogeneity of non-criteria APS manifestations and the diversity in management approaches. Conventional anticoagulation and/or antiplatelet APS treatment do not adequately control most of non-criteria manifestations. Increasing knowledge about the contribution of inflammatory in addition to, or independently of, thrombotic mechanisms in non-criteria APS manifestations provides insight into the potential effect of novel therapies targeting B-cells, mammalian target of rapamycin, neutrophil, and complement or interferon pathways. Existing evidence is limited by lack of high-quality studies. Better understanding of the pathophysiology and clinical phenotypes of APS and well-designed prospective studies of homogenous populations are needed to provide evidence-based recommendations for the management of non-criteria APS manifestations.