-
Best Practice & Research. Clinical... Feb 2020Antiphospholipid syndrome is an autoimmune systemic disorder characterized by arterial, venous, or small vessel thrombosis and/or recurrent early pregnancy loss, fetal... (Review)
Review
Antiphospholipid 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, thrombocytopenia, hemolytic anemia, valvular heart disease, and nephropathy. The degree of risk associated with antiphospholipid antibody depends on the characteristics of the antiphospholipid antibody profile and on the presence of additional thrombotic risk factors. Current standard treatment for unprovoked thrombosis is long-term warfarin or other vitamin K antagonist therapy. Treatment to prevent recurrent obstetric complications is low-dose aspirin and prophylactic heparin, usually low-molecular-weight heparin. Optimal treatment for standard therapy failures or for certain nonthrombotic manifestations is uncertain, although nonanticoagulation therapies that address multiple demonstrated mechanisms of disease are being explored.
Topics: Abortion, Habitual; Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Embryo Loss; Female; Humans; Lupus Coagulation Inhibitor; Pregnancy
PubMed: 31866276
DOI: 10.1016/j.berh.2019.101463 -
Translational Research : the Journal of... Nov 2020Antiphospholipid syndrome is one of the more common acquired causes of hypercoagulability. Its major presentations are thrombotic (arterial, venous, or microvascular)... (Review)
Review
Antiphospholipid syndrome is one of the more common acquired causes of hypercoagulability. Its major presentations are thrombotic (arterial, venous, or microvascular) and pregnancy morbidity (miscarriages, late intrauterine fetal demise, and severe pre-eclampsia). Classification criteria include 3 different antiphospholipid antibodies: lupus anticoagulant, anticardiolipin, and anti-beta 2 glycoprotein I. Management includes both preventive strategies (low-dose aspirin, hydroxychloroquine) and long-term anticoagulation after thrombosis.
Topics: Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Humans
PubMed: 32413497
DOI: 10.1016/j.trsl.2020.04.006 -
The New England Journal of Medicine May 2018
Review
Topics: Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Humans; Thrombosis; Warfarin
PubMed: 29791828
DOI: 10.1056/NEJMra1705454 -
Lancet (London, England) Oct 2010The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or... (Review)
Review
The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs.
Topics: Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Complications, Hematologic; Thrombosis
PubMed: 20822807
DOI: 10.1016/S0140-6736(10)60709-X -
Frontiers in Immunology 2019
Topics: Antiphospholipid Syndrome; Diagnosis, Differential; Disease Susceptibility; Humans; Lupus Erythematosus, Systemic
PubMed: 30858846
DOI: 10.3389/fimmu.2019.00199 -
Thrombosis Research Sep 2019Antiphospholipid syndrome (APS) is an autoimmune disease characterised by the presence of antiphospholipid antibodies (aPL). The antibodies currently included in the... (Review)
Review
Antiphospholipid syndrome (APS) is an autoimmune disease characterised by the presence of antiphospholipid antibodies (aPL). The antibodies currently included in the classification criteria include lupus anticoagulant (LA), anticardiolipin antibodies (aCL) and anti-^2-glycoprotein 1 antibodies (^2GPI). APS can present with a variety of clinical phenotypes, including thrombosis in the veins, arteries and microvasculature and obstetrical complications. Pregnancy complications in obstetric APS (OAPS) include unexplained recurrent early pregnancy loss, fetal death, or premature birth due to severe preeclampsia, eclampsia, intrauterine growth restriction or other consequences of placental insufficiency. Careful, well monitored obstetric care with the use of aspirin and heparin has likely improved the pregnancy outcome in obstetric APS and currently approximately 70-80% of pregnant women with APS have a successful pregnancy outcome. However, the current standard of care does not prevent all pregnancy complications as the current treatment fails in 20-30% of APS pregnancies. Other treatments options are currently being explored and retrospective studies suggest that trials with hydroxychloroquine and possibly pravastatin are warranted in pregnant women with aPL. In this review will focus on the current treatment of OAPS.
Topics: Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Outcome
PubMed: 31477227
DOI: 10.1016/S0049-3848(19)30366-4 -
Expert Review of Hematology Apr 2017Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the... (Review)
Review
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia. Expert commentary: CAPS is a rare variant of the antiphospholipid syndrome (APS). It is characterized by thrombosis in multiple organs and a cytokine storm developing over a short period, with histopathologic evidence of multiple microthromboses, and laboratory confirmation of high aPL titers. This review discusses the diagnostic challenges and current approaches to the treatment of CAPS.
Topics: Algorithms; Antiphospholipid Syndrome; Combined Modality Therapy; Diagnosis, Differential; Disease Management; Humans; Phenotype; Prognosis; Risk Factors; Treatment Outcome
PubMed: 28277850
DOI: 10.1080/17474086.2017.1300522 -
Nature Reviews. Disease Primers Jan 2018
-
Kidney & Blood Pressure Research 2023Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the development of autoantibodies and the impairment of the coagulation system. Knowledge about... (Review)
Review
BACKGROUND
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the development of autoantibodies and the impairment of the coagulation system. Knowledge about this syndrome is increasing over time, but kidney involvement, especially APS nephropathy, still represents a challenge for physicians.
SUMMARY
A "two hit" model has been hypothesized to explain APS pathophysiology, and the role played by some factors, such as the complement system, is becoming more and more clear. From a clinical point of view, along with thrombosis in any site and/or obstetric morbidities, that are the hallmarks of APS, a constellation of several other clinical symptoms is related to APS. These symptoms alone are not sufficient to fulfill Sydney criteria for APS and this could potentially lead to omitting some diagnoses. The mainstay of management of APS is antithrombotic therapy, but there are expectations for new drugs that regulate the immune system. APS could affect the kidneys in many ways and among them, APS nephropathy is an intriguing entity that has been overlooked in recent years. Novel studies on APS nephropathy are lacking.
KEY MESSAGES
In this review, we discuss what we currently know about APS and its relationship with the kidney, with an eye toward the future perspectives. Multicenter studies on APS nephropathy are necessary in order to develop targeted therapies.
Topics: Female; Pregnancy; Humans; Antiphospholipid Syndrome; Antibodies, Antiphospholipid; Kidney; Kidney Diseases; Thrombosis
PubMed: 37734329
DOI: 10.1159/000529229 -
European Journal of Haematology Dec 2023Antiphospholipid syndrome is an autoimmune disorder which is characterized by the presence of heterogeneous antiphospholipid antibodies. There is an evidence on... (Review)
Review
Antiphospholipid syndrome is an autoimmune disorder which is characterized by the presence of heterogeneous antiphospholipid antibodies. There is an evidence on antiphospholipid (aPL) antibodies related to thromboembolic events in cancer patients. In fact, the thrombotic complications in patients with malignancy occur at a rather high frequency, compared to other risk factors. In parallel with standard therapies available, there is need of case-by-case monitoring of each patient and the introduction of new therapies and need for more clinical trials which will address many questions for the optimal management of patients. This paper presents a basic review of the literature on the aPL antibodies associated with cardiovascular disease and cancer, as well as its complications, which are reported so far in the bibliography.
Topics: Humans; Antiphospholipid Syndrome; Cardiovascular Diseases; Antibodies, Antiphospholipid; Autoimmune Diseases; Neoplasms
PubMed: 37667555
DOI: 10.1111/ejh.14096