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Primary antiphospholipid syndrome

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TLDR
Primary APS occurs when there is no evidence of associated diseases, and APS in the presence of an underlying disease, usually systemic lupus erythematosus, is called secondary APS.
Abstract
Antiphospholipid syndrome (APS) is a disorder characterised by recurrent arterial or venous thrombosis and/or pregnancy losses, in the presence of persistently elevated levels of anticardiolipin antibodies and/or evidence of circulating lupus anticoagulant (these abnormalities are detected by blood tests). Primary APS occurs when there is no evidence of associated diseases. APS in the presence of an underlying disease, usually systemic lupus erythematosus, is called secondary APS.

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Citations
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Journal ArticleDOI

The antiphospholipid syndrome: from pathophysiology to treatment

TL;DR: The objective of this review paper is to summarize the recent literature on APS from pathogenesis to current therapeutic options.
Journal ArticleDOI

Microvascular and microangiopathic antiphospholipid-associated syndromes ("MAPS"): semantic or antisemantic?

TL;DR: Small vessel occlusions may occur as part of the vascular manifestations of the Antiphospholipid Syndrome and may affect glomerular, skin, retinal, bowel, hepatic or pulmonary vessels, and the term "MAPS" is suggested for these two groups of conditions.
Journal ArticleDOI

Isolated IgA anti- β2 glycoprotein I antibodies in patients with clinical criteria for antiphospholipid syndrome.

TL;DR: Assessment of aPL antibodies in a group of patients with clinical manifestations of APS (C-APS) to evaluate the importance of the presence of IgA aB2GPI antibodies in APS and its relation with other a-PL antibodies.
Journal ArticleDOI

Antiphospholipid syndrome and systemic lupus erythematosus: are they separate entities or just clinical presentations on the same scale?

TL;DR: A multiorgan involvement that cannot be explained by the thrombophilic state per se and similar to the well known clinical manifestations in lupus is reported in patients with primary antiphospholipid syndrome.
Journal ArticleDOI

Is there a microangiopathic antiphospholipid syndrome

TL;DR: Although large-vessel occlusions do occur in catastrophic APS, they do not dominate the clinical picture, and their frequency is completely different from that encountered in the classic APS itself.
References
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Journal ArticleDOI

Catastrophic antiphospholipid syndrome associated with malignancies (case report and review of the literature).

TL;DR: The case indicates that there is an overlap between antiphospholipid syndrome (APS) and Trousseau's syndrome, and it is important to bear in mind that a thrombotic event associated with cancer can be the first manifestation of CAPS.
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Identifying phenotypes of patients with antiphospholipid antibodies: results from a cluster analysis in a large cohort of patients

TL;DR: While clusters 1, 2, 3 and 5 corresponded to well-known entities, cluster 4 might represent a bridging condition between purePrimary APS and defined SLE, with lower thrombotic risk when compared with primary APS but higher general features such as ANA and cytopenia (mainly throm bocy topenia).
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The paradox of the lupus anticoagulant: history and perspectives.

TL;DR: A unique coagulation inhibitor prolonging whole-blood clotting time was described more than 50 years ago in two patients with systemic lupus erythematosus (SLE) and its interaction with antiphospholipid antibodies was later demonstrated as discussed by the authors.
Journal ArticleDOI

Antiphospholipid Antibodies: Laboratory and Pathogenetic Aspects

TL;DR: Clinical and experimental data have shown that aPL exert their pathogenic activity by interfering with the function of coagulation factors, such as thrombin and factors X, XI and XII, and with thefunction of anticoagulant proteins of the protein C system.
Journal ArticleDOI

Antiphospholipid Syndrome and Renal Allograft Thrombosis.

TL;DR: A new form of APS based on IgA anti-β-2-glycoprotein-I (B2GPI) antibodies, representing up to 30% of patients in end-stage renal disease and renal transplantation, is the main independent risk factor for graft thrombosis and early graft loss after renal transplation.
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