Management of Non-criteria Manifestations in Antiphospholipid Syndrome
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ANTIPHOSPHOLIPID SYNDROME (S ZUILY, SECTION EDITOR)
Management of Non-criteria Manifestations in Antiphospholipid Syndrome Eleni Xourgia 1 & Maria G. Tektonidou 2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
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. Keywords Non-criteria antiphospholipid syndrome manifestations . Heart valve disease . Diffuse alveolar hemorrhage . Antiphospholipid syndrome nephropathy . Thrombocytopenia . Skin ulcers
Introduction A number of heart, pulmonary, hematological, renal, skin, and neurological antiphospholipid antibody (aPL)-related manifestations have been considered as common but not specific for antiphospholipid syndrome (APS) by the updated Sapporo classification criteria [1], defined as non-criteria APS manifestations. A task force on non-criteria manifestations took place in the 14th International Congress on aPL to evaluate the This article is part of the Topical Collection on Antiphospholipid Syndrome * Maria G. Tektonidou [email protected]; [email protected] 1
First Department of Propaedeutic Internal Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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
quality of evidence for the outcomes linked to each of these manifestations and whether it supports their inclusion in the classification criteria for APS [2]. The overall quality of evidence for heart valve lesions, APS nephropathy, and livedo reticularis was graded as moderate, while for superficial venous thrombosis, thrombocytopenia, chorea, and longitudinal myelitis was considered low. Anticoagulation the
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