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Management of Non-criteria Manifestations in Antiphospholipid Syndrome

  • Antiphospholipid Syndrome (S Zuily, Section Editor)
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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.

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  1. First Department of Propaedeutic Internal Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece

    Eleni Xourgia

  2. First Department of Propaedeutic Internal Medicine, Joint Rheumatology program, Medical School, ‘Laiko’ Hospital, National and Kapodistrian University of Athens, 17 Agiou Thoma Street., 11527, Athens, Greece

    Maria G. Tektonidou

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Xourgia, E., Tektonidou, M.G. Management of Non-criteria Manifestations in Antiphospholipid Syndrome. Curr Rheumatol Rep 22, 51 (2020). https://doi.org/10.1007/s11926-020-00935-2

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