Treatment of thrombotic antiphospholipid syndrome in adults and children

Purpose of review Antiphospholipid syndrome (APS), more common than once believed, is an autoimmune disease best known for its high risk of incident and recurrent thrombotic events. The approach to treatment potentially differs from treatment of thrombosis in the general population, and this article endeavors to review the latest updates on this topic. Recent findings The epidemiology of APS is being increasingly elucidated by large population-based studies, with APS perhaps affecting as many as 1 in 2000 individuals. Vitamin K antagonists, aspirin, and heparinoids continue to have obvious roles in the management of patients with APS. There has recently been intensive study of direct oral anticoagulants in APS, with the most recent randomized studies raising concerns about their inferiority to vitamin K antagonists, at least in some subgroups. Other approaches to treating APS beyond anticoagulants and antiaggregants are also receiving increased attention in mechanistic and preclinical studies with an eye toward future roles in patients with refractory and/or microvascular disease. Pediatric APS is identified as an area in desperate need of additional prospective research. Summary Progress continues to be made in pursuit of improving the lives of individuals afflicted with APS. The most important future directions would seem to involve leveraging modern molecular technologies in order to improve subphenotyping of antiphospholipid antibody-positive individuals. This wil...
Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: CLINICAL THERAPEUTICS AND HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune Source Type: research

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AbstractPurpose of ReviewAntiphospholipid syndrome (APS) is a chronic autoimmune disease that can be seen as a burden, with consequences on patients ’ daily life. Health has traditionally been measured using measures of morbidity or mortality. Health-related quality of life (HRQoL) is a concept that includes quality of life through physical, mental, and social domains. As in other autoimmune diseases, HRQoL has been investigated in patients wi th APS. Here, we provide a comprehensive review of the current knowledge of the assessment of HRQoL in APS.Recent FindingsAPS patients have an impaired HRQoL compared with the ...
Source: Current Rheumatology Reports - Category: Rheumatology Source Type: research
Authors: Salle V Abstract The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis and/or obstetrical manifestations and the persistent presence, at least 12 weeks apart, of antiphospholipid antibodies (aPL) such as lupus anticoagulant (LA) and/or anticardiolipin antibodies (ACL) and/or anti-β2 glycoprotein I antibodies (aβ2GPI). The finding of patients with clinical profile highly suggestive of APS but who are negative for conventional biological criteria has led to the concept of seronegative APS. In the last few years, new antigen targets and methodological appro...
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
Conclusion Coagsense correlated better than Coagucheck XS and did not show increasing bias as INR increased. Both POC instruments had higher INR variability in 4 disease states (antiphospholipid syndrome, autoimmune, peripheral vascular disease, and hypercoagulable). Patient-specific laboratory correlations may be needed on each POC device.
Source: Point of Care: The Journal of Near-Patient Testing and Technology - Category: Pathology Tags: Original Articles Source Type: research
CONCLUSION: The incidence of APS differs according to age groups and gender. The incidence of primary APS was higher than that of secondary APS in both gender. Furthermore, as already reported, secondary APS is highly associated with SLE; however, we observed that rheumatoid arthritis is also highly related. PMID: 32030922 [PubMed - in process]
Source: Journal of Korean Medical Science - Category: Biomedical Science Tags: J Korean Med Sci Source Type: research
Abstract Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and venous thrombotic manifestations and/or pregnancy-related complications in patients with persistently high antiphospholipid antibodies (aPL), the most common being represented by anticardiolipin antibodies (aCL), anti-beta 2 glycoprotein-I (aβ2GPI) and lupus anticoagulant (LAC). A growing number of studies showed that in some cases patients may present with clinical features of APS but with temporary positive or persistently negative titers of aPL. For these patients, the definition of seronegative APS (SN-...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
Yue Lang, Weiguanliu Zhang, Xiujuan Wu, Fang Deng, Li CuiAnnals of Indian Academy of Neurology 2020 23(1):110-112 Cerebral venous sinus thrombosis (CVST) is a cerebrovascular disease that is caused by a number of factors, including hypercoagulability and vessel wall damage. Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocyte infiltration of the exocrine glands. CVST could be caused by autoimmune diseases. According to previous reports, the most frequently reported autoimmune diseases which could cause CVST are systemic lupus erythematosus and antiphospholipid syndrome....
Source: Annals of Indian Academy of Neurology - Category: Neurology Authors: Source Type: research
Authors: Wang D, Lv W, Zhang S, Zhang J Abstract Anticardiolipin antibody (ACA) is a kind of autoantibody and is one of the antiphospholipid antibodies (aPLs). Phospholipids with a negative charge on platelets and endothelial cell membranes are ACA target antigens. ACA is common in systemic lupus erythematosus and other autoimmune diseases and is closely associated with thrombosis, thrombocytopenia, and spontaneous abortion. In 1983, Harris established a method for detecting ACA, and research on the antibody has gained worldwide attention and has developed rapidly. For this review, we browsed articles that cover mo...
Source: Journal of Immunology Research - Category: Allergy & Immunology Tags: J Immunol Res Source Type: research
Publication date: Available online 19 December 2019Source: Best Practice &Research Clinical RheumatologyAuthor(s): Lisa R. SammaritanoAbstractAntiphospholipid syndrome is an autoimmune systemic disorder characterized by arterial, venous, or small vessel thrombosis and/or recurrent early pregnancy loss, fetal loss, or pregnancy morbidity in the setting of documented persistent antiphospholipid antibodies that include the lupus anticoagulant, or moderate-high titer anticardiolipin, or anti-β2Glycoprotein I antibodies. Associated clinical manifestations include livedo reticularis, cutaneous ulcerations, thrombocytope...
Source: Best Practice and Research Clinical Rheumatology - Category: Rheumatology Source Type: research
Semin Thromb Hemost DOI: 10.1055/s-0039-3400258Primary hemostasis, similar to other systems in the adjusting and transitioning neonate, undergoes developmental adaptations in the first days of life. Although platelets of neonates do not differ quantitatively compared with those of adults, they functionally present with major differences, thus supporting the theory of a “hypofunctional” phenotype that is counterbalanced by high hematocrit and more potent von Willebrand factor multimers. No clinical effect of bleeding tendency has hence been established so far for healthy term neonates. However, discrepancies in ...
Source: Seminars in Thrombosis and Hemostasis - Category: Hematology Authors: Tags: Review Article Source Type: research
Authors: Sevim E, Willis R, Erkan D Abstract Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombosis, pregnancy morbidity, or nonthrombotic manifestations in patients with persistently positive antiphospholipid antibodies (aPL). Conventional APS treatment focuses on antithrombotic strategies, which are usually ineffective for the microvascular and nonthrombotic manifestations of aPL. Using a case-based presentation, this review focuses on the role of immunosuppression in nonobstetric APS, including B-cell inhibition (rituximab, belimumab, and bortezomib), complement inhibition ...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
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