Published in:
01-09-2020 | Antiphospholipid Syndrome | Antiphospholipid Syndrome (S Zuily, Section Editor)
Management of Non-criteria Manifestations in Antiphospholipid Syndrome
Authors:
Eleni Xourgia, Maria G. Tektonidou
Published in:
Current Rheumatology Reports
|
Issue 9/2020
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Abstract
Purpose of the Review
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.
Recent Findings
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.
Summary
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.