Primary Antiphospholipid Syndrome

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Background

Antiphospholipid syndrome (APS) is defined by the presence of thrombosis or gestational morbidity in people harboring anti-phospholipid antibodies. APS was initially described in patients with concomitant autoimmune diseases, such as Systemic Lupus Erythematosus (SLE), and is widely studied in this context. Shortly after APS was described, it was found that it could also appear in patients with no preceding history of autoimmune disease. This was defined as the primary form of APS (P-APS). Therefore, two basic forms of APS were subsequently established: P-APS and APS related to other systemic autoimmune diseases (SAD-APS).

The epidemiology, pathogenesis and characteristics of P-APS are much less characterized in comparison with SAD-APS. This is since, in the case of SAD-APS, both clinical and analytical data (and even stored serum samples), are available prior to the onset of the syndrome. However, P-APS is an entity that can appear sporadically in people with no previous history of disease. Generally, P-APS diagnosis is made only after ruling out other possible causes and many patients remain un-diagnosed. In recent years, P-APS, outside the context of systemic autoimmune diseases, is being diagnosed at an increasing frequency in both pre-symptomatic patients and in patients with other chronic non-autoimmune diseases (e.g. renal and cardiac failure).

The aim of this Research Topic is to gather a collection of basic and translational research studies and the most recent clinical studies into P-APS in order to (i) help unravel the patho-physiological basis of this disease; (ii) define new diagnostic and therapeutic tools for this disease and (iii) identify patients that are at risk of developing APS in order to enable the application of preventive measures in the context of predictive and anticipatory medicine. In this Research Topic, we welcome the submission of Original Research, Reviews, Mini-Reviews, Perspective, Opinion, Clinical Trial, Case Reports, Methods and Protocols that cover the following topics:

1. Epidemiology and etiology of Primary APS (not related with systemic autoimmune diseases).
2. Primary APS diagnosis.
3. Infection and APS.
4. Immunomodulators in APS.
5. The role of immune cells and cytokines in APS.
6. APS and organ transplantation.
7. New biomarkers for APS.
8. Atherosclerosis and APS.
9. Catastrophic APS.
10. APS and pregnancy.
11. APS in the context of renal and hepatic failure.
12. Cardiac affections and APS.
13. Nervous system and APS.
14. New therapeutic approaches to non-SAD APS.

Keywords: Antiphospholipid syndrome, APS, Antiphospholipid, thrombosis, cardiolipin, autoimmunity

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