Antiphospholipid Syndrome: A Review

2018 ◽  
Vol 2 (02) ◽  
pp. 51-58
Author(s):  
Md. Motahar Hossain ◽  
Md. Akhter Hossain ◽  
Yasmin Rahman ◽  
Md. Kamrul Hasan
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Anticardiolipin Antibodies ◽  
Vitamin K Antagonist ◽  
Classification Criteria ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Pregnancy Morbidity ◽  
Pregnancy And Postpartum

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by venous thromboembolism, arterial thrombosis, and obstetric morbidities in the setting of persistently positive levels of antiphospholipid antibodies. It may be primary or secondary. The latest classification criteria (Sydney 2006) recognize just three tests to define this syndrome- lupus anticoagulant, anticardiolipin antibodies and anti-?2-glycoprotein-1 antibodies. Treatment of thrombotic events involves lifelong anticoagulation with vitamin K antagonist warfarin. Antiphospholipid antibody syndrome (APS) with only pregnancy morbidity is treated with thromboprophylaxis with heparin during pregnancy and postpartum for 6 weeks. In this review we discuss the pathogenesis, diagnosis, treatment and prognosis of the APS.

Antiphospholipid Antibody Syndrome

2011 ◽  
Vol 135 (9) ◽  
pp. 1092-1096 ◽  
Author(s):  
Nikhil A Sangle ◽  
Kristi J Smock
Keyword(s):  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Protein Complexes ◽  
Anticardiolipin Antibodies ◽  
Clinical Aspects ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Pregnancy Morbidity ◽  
Glycoprotein I ◽  
Laboratory Results

Antiphospholipid antibodies are directed against phospholipid-protein complexes and include lupus anticoagulant, anticardiolipin antibodies, and anti–beta-2 glycoprotein I antibodies. Antiphospholipid antibody syndrome is a common cause of acquired thrombophilia and is characterized by venous or arterial thromboembolism or pregnancy morbidity and the presence of antiphospholipid antibodies. Antibodies should be demonstrable on at least 2 occasions separated by 12 weeks. Heterogeneity of the autoantibodies and absence of gold standard assays makes interpretation of laboratory results a challenge for both laboratorians and clinicians. This review discusses the key laboratory and clinical aspects of antiphospholipid antibody syndrome. Particular focus is given to lupus anticoagulant detection, in view of recently updated laboratory guidelines.

IgA Anticardiolipin Antibodies – Relation with other Antiphospholipid Antibodies and Clinical Significance

1998 ◽  
Vol 79 (02) ◽  
pp. 282-285 ◽  
Author(s):  
Josep Ordi-Ros ◽  
Francesc Monegal-Ferran ◽  
Nuria Martinez ◽  
Fina Cortes-Hernandez ◽  
Miquel Vilardell-Tarres ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Fetal Loss ◽  
Cross Sectional Study ◽  
Anticardiolipin Antibodies ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Serum Samples ◽  
Cross Sectional ◽  
Vein Thrombosis

SummaryObjective: To evaluate the usefulness of IgA antiphospholipid antibodies as markers of thrombosis and/or antiphospholipid antibody syndrome. Patients and Methods: A cross-sectional study design in a tertiary, university-based, autoimmune reference hospital. Seven-hundred ninety-five patients classified into five different groups – autoimmune diseases (255), deep vein thrombosis (153), transitory ischemic attacks (108), obstetric complications (196), infectious diseases (83) and controls (81) – were tested for IgA, IgG and IgM aPL, and lupus anticoagulant. Plasma and serum samples were drawn for detection of aPL using an internationally standardized ELISA method and LA was carried out using coagulometric assays. Results: True IgA aPL were found only in two patients with systemic lupus erythematosus; these patients were also positive to IgG aPL. Conclusion: The incidence of true positivity to IgA anticardiolipin antibodies is extremely low. Their determination was not helpful in diagnosing the antiphospholipid syndrome or in explaining thrombotic events or aPL related manifestations – fetal loss – in the groups studied.

The antiphospholipid (antibody) syndrome

2011 ◽  
pp. 343-352
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Pregnancy Morbidity

Introduction 344 Epidemiology and pathology 345 Clinical features of antiphospholipid syndrome 346 Treatment of antiphospholipid syndrome 348 Catastrophic antiphospholipid syndrome 350 The antiphospholipid syndrome (APS) was first described in the 1980s and comprises arterial and venous thrombosis with or without pregnancy morbidity in the presence of anticardiolipin (ACL) antibodies or the lupus anticoagulant (LAC). It can be primary, or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE) (...

scholarly journals Epidemiological, clinical and immune factors that influence the persistence of antiphospholipid antibodies in leprosy

2019 ◽  
Vol 59 (1) ◽  
Author(s):  
Sandra Lúcia Euzébio Ribeiro ◽  
Helena Lúcia Alves Pereira ◽  
Antonio Luiz Boechat ◽  
Neusa Pereira Silva ◽  
Emilia Ionue Sato ◽  
...  
Keyword(s):  
Antiphospholipid Antibodies ◽  
Specific Treatment ◽  
Binary Logistic Regression ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Pregnancy Morbidity ◽  
Glycoprotein I ◽  
Leprosy Patients ◽  
Immunological Factors ◽  
Bacterial Index

Abstract Introduction Antiphospholipid antibodies (aPL) are described in individuals with leprosy without the clinical features of antiphospholipid antibody syndrome (APS), a condition involving thromboembolic phenomena. We have described the persistence of these antibodies for over 5 years in patients with leprosy after specific treatment. Objectives To determine whether epidemiological, clinical and immunological factors played a role in the long-term persistence of aPL antibodies in leprosy patients after multidrug therapy (MDT) had finished. Methods The study sample consisted of 38 patients with a diagnosis of leprosy being followed up at the Dermatology and Venereology Outpatient Department at the Alfredo da Matta Foundation (FUAM) in Manaus, AM. ELISA was used to detect anticardiolipin (aCL) and anti-β2 glycoprotein I (anti-β2GPI) antibodies. Patients were reassessed on average of 5 years after specific treatment for the disease (MDT) had been completed. Results Persistence of aPL antibodies among the 38 leprosy patients was 84% (32/38), and all had the IgM isotype. Mean age was 48.1 ± 15.9 years, and 23 (72.0%) were male. The lepromatous form (LL) of leprosy was the most common (n = 16, 50%). Reactional episodes were observed in three patients (9.4%). Eighteen (47.37%) were still taking medication (prednisone and/or thalidomide). Mean IgM levels were 64 U/mL for aCL and 62 U/mL for anti-β2GPI. In the multivariate binary logistic regression the following variables showed a significant association: age (p = 0.045, OR = 0.91 and CI 95% 0.82–0.98), LL clinical presention (p = 0.034; OR = 0.02 and CI 95% = 0.0–0.76) and bacterial index (p = 0.044; OR = 2.74 and CI 95% = 1.03–7.33). We did not find association between prednisone or thalidomide doses and positivity for aPL (p = 0.504 and p = 0.670, respectively). No differences in the variables vascular thrombosis, pregnancy morbidity, diabetes, smoking and alcoholism were found between aPL-positive and aPL-negative patients. Conclusion Persistence of positivity for aPL antibodies was influenced by age, clinical presentation and bacterial index. However, further studies are needed to elucidate the reason for this persistence, the role played by aPL antibodies in the disease and the B cell lineages responsible for generation of these antibodies.

scholarly journals Role of tissue factor in thrombosis in antiphospholipid antibody syndrome

Lupus ◽  
2010 ◽  
Vol 19 (4) ◽  
pp. 370-378 ◽  
Author(s):  
J. Boles ◽  
N. Mackman
Keyword(s):  
Tissue Factor ◽  
Mouse Models ◽  
Arterial Thrombosis ◽  
Autoimmune Disorder ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Pregnancy Morbidity ◽  
Prothrombotic Effect

Antiphospholipid syndrome (APS) is an acquired autoimmune disorder defined by the presence of an antiphospholipid antibody (aPL) and the occurrence of at least one associated clinical condition that includes venous thrombosis, arterial thrombosis or pregnancy morbidity. The aPL detected in APS have long been thought to have a direct prothrombotic effect in vivo. However, the pathophysiology underlying their coagulopathic effect has not been defined. Emerging data suggest a role for the procoagulant protein tissue factor (TF). In this review we provide an overview of TF, describe mouse models used in the evaluation of the role of TF in thrombosis, as well as summarize recent work on TF and APS. Lupus (2010) 19, 370—378.

Antiphospholipid syndrome

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 675-680 ◽  
Author(s):  
Wendy Lim
Keyword(s):  
Antiphospholipid Syndrome ◽  
Arterial Thrombosis ◽  
Rapid Development ◽  
Multiorgan Failure ◽  
Severe Form ◽  
Anticardiolipin Antibodies ◽  
Recurrent Thrombosis ◽  
Thrombotic Risk ◽  
Pregnancy Morbidity ◽  
Increased Risk

Abstract The antiphospholipid syndrome (APS) is defined by venous or arterial thrombosis and/or pregnancy morbidity in patients with persistent presence of antiphospholipid antibodies (aPLs). Catastrophic APS is the most severe form of APS, which is associated with rapid development of microvascular thrombosis resulting in multiorgan failure in patients with aPLs. Patients with APS and catastrophic APS are recognized to have a high risk of recurrent thrombosis that can occur despite anticoagulant therapy. Although antithrombotic therapy remains the mainstay of treatment, bleeding manifestations can complicate management and contribute to increased morbidity. Patients with persistently elevated aPL levels, particularly those who exhibit positive testing for lupus anticoagulant, anticardiolipin antibodies, and anti-β2GPI antibodies (triple positivity), appear to be at increased risk for thrombosis and pregnancy complications, whereas isolated positivity for aPLs appears to be associated with low risk. Recognizing that patients with APS have different thrombotic risk profiles may assist clinicians in assessing the risks and benefits of anticoagulation. The optimal type, intensity, and duration of anticoagulation in the treatment of APS remain controversial, particularly for arterial thrombosis and recurrent thrombosis. Future studies that delineate thrombotic risk in APS and evaluate current and novel anticoagulants as well as nonanticoagulant therapies are required.

CLINICAL SIGNIFICANCE OF APTT - ANTIPHOSPHOLIPID ANTIBODY SYNDROME PRESENTING AS STROKE.

2021 ◽  
pp. 1-2
Author(s):  
Dinesh Chaudhari ◽  
Ramesh Krishnan ◽  
Pushpendra N Renjen ◽  
Shishir Seth
Keyword(s):  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Sudden Onset ◽  
Clinical Suspicion ◽  
Systemic Autoimmune Disease ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
High Clinical Suspicion ◽  
Treatable Condition

The antiphospholipid syndrome is a systemic autoimmune disease dened by thrombotic or obstetrical events that occur in patients with persistent antiphospholipid antibodies. Anti-phospholipid antibodies (APLA) are a part of heterogeneous group of circulating serum polyclonal immunoglobulins (IgG, IgM, IgA or mixed) that bind negatively charged or neutral phospholipid component of cell membranes and cause increased tendency to venous or arterial thrombosis. Persistently positive APS requires that laboratory tests be conducted at least 12 weeks apart. We report a 67 years old pleasant lady presented with sudden onset dizziness, left sided weakness and right facial deviation for two days. In view of deranged aPTT and high clinical suspicion, Lupus anticoagulation tests were done using DRVVT which was prolonged. Tests were repeated after 12 weeks and Lupus anticoagulant remained positive. Routine aPTT detects upto 30% of APLAS, but DRVVT and Antibodies must be considered in such cases. This is a treatable condition, and can be prevented with long term anticoagulation. Key Message- Routine aPTT detects upto 30% of APLAS, but DRVVT and Antibodies must be considered in such cases. This is a treatable condition, and can be prevented with long term anticoagulation.

scholarly journals ANTIPHOSPHOLIPID SYNDROME

2017 ◽  
pp. 4-11
Author(s):  
E. V. Makarenko
Keyword(s):  
Blood Plasma ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Lupus Anticoagulant ◽  
Pregnancy Complication ◽  
Classification Criteria ◽  
Clinical Criteria ◽  
Glycoprotein I ◽  
Acquired Thrombophilia

Antiphospholipid syndrome is autoimmune acquired thrombophilia associated with the formation of antibodies to phospholipids, which is manifested by recurrent venous or arterial thrombosis and/or pathology of pregnancy. Antiphospholipid antibodies are a heterogeneous group of autoantibodies interacting with phospholipids, which are components of cell membranes and phospholipid-binding proteins of blood plasma. Antiphospholipid syndrome can affect vessels of any caliber and localization, with thrombosis accompanied by no morphological signs of inflammation in the wall of the vessel. Obstetrical pathology is manifested by loss of the fetus, which can occur at any time of pregnancy, as well as other complications of pregnancy, such as preeclampsia and placental insufficiency. Based on the classification criteria, antiphospholipid syndrome is diagnosed if one of the clinical criteria (thrombosis or pregnancy complication) and one of the laboratory criteria including the lupus anticoagulant, antibodies to cardiolipin or β2-glycoprotein I, are revealed. The main tactic of the treatment of patients with antiphospholipid syndrome is to prevent thrombosis. For this purpose, the traditional therapy with anticoagulants and antiaggregants is applied. In addition, new medicines are being developed and evaluated

scholarly journals The effect of low dose aspirin and low molecular weight heparin (enoxaparin) in recurrent pregnancy loss associated with antiphospholipid antibody syndrome

2017 ◽  
Vol 6 (11) ◽  
pp. 4830 ◽  
Author(s):  
Sasmita Swain ◽  
Sujata Singh
Keyword(s):  
Antiphospholipid Syndrome ◽  
Live Birth ◽  
Antiphospholipid Antibodies ◽  
Pregnancy Loss ◽  
Low Dose ◽  
Recurrent Pregnancy Loss ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Dose Aspirin ◽  
Low Dose Aspirin

Background: Recurrent miscarriage affects 1–2% of women. Recurrent pregnancy loss (RPL) is the loss of three or more consecutive pregnancies before or during the 20th week of gestation. The most important association between gestational loss and autoimmune phenomena is the presence of antiphospholipid antibodies represented by the lupus anticoagulants and or anticardiolipin antibodies (Antiphospholipid Antibody Syndrome). The antiphospholipid syndrome is an acquired autoimmune. The clinical features are thrombosis (venous, arterial and microvascular) and/or pregnancy complications; the most prominent of which is recurrent abortion.Methods: Twenty-two selected patients during pregnancy with clinical and/or serological findings of antiphospholipid syndrome had received low dose aspirin (75 mg once daily orally) plus LMWH enoxaparin (40 mg subcutaneously/day).Results: There are live born in 86% cases compared to abortion (< 20 weeks gestational age) in 14 % cases. From 19 liveborn babies the mother having normotensive in 79% and preeclampsia 21%, 85% babies having normal growth and 15% are IUGR. 36% cases are at term (>37 weeks) and 50% cases are at preterm (<37 week) on which 9%) is spontaneous preterm and 41% is iatrogenic preterm due to preeclampsia, IUGR, PPROM and APH.Conclusions: Use of low dose aspirin (75mg) and enoxaparin 40 mg subcutaneously daily in patients with RPL due to antiphospholipid syndrome resulted in higher live birth rates. Combination treatment with aspirin and LMWH leads to a high live birth rate among women with recurrent abortion and antiphospholipid antibodies. 

scholarly journals Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome

2021 ◽  
Vol 10 (6) ◽  
pp. 1240
Author(s):  
Takako Miyamae ◽  
Tomohiro Kawabe
Keyword(s):  
Heart Disease ◽  
Antiphospholipid Syndrome ◽  
Valvular Heart Disease ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Autoimmune Disorder ◽  
Classification Criteria ◽  
Neurologic Manifestations ◽  
Test Results ◽  
Pregnancy Morbidity

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder mainly characterised by increased risks of thrombosis and pregnancy morbidity and persistent positive test results for antiphospholipid antibodies (aPLs). The criteria for diagnosing juvenile APS have yet to be validated, while the Sydney classification criteria do not contain several non-thrombotic clinical manifestations associated with the presence of aPLs. As such, difficulties have been encountered in the diagnosis of patients who have no certain thrombotic occlusions. Moreover, extra-criteria manifestations (i.e., clinical manifestations not listed in the classification criteria), including neurologic manifestations (chorea, myelitis and migraine), haematologic manifestations (thrombocytopenia and haemolytic anaemia), livedo reticularis, nephropathy and valvular heart disease have been reported, which suggests that the clinical spectrum of aPL-related manifestations extends beyond that indicated in the classification criteria. Studies have demonstrated that more than 40% of children with aPLs demonstrated non-thrombotic aPL-related clinical manifestations alone. Moreover, our results showed that the pathogenesis of non-criteria manifestations is characterised by “APS vasculopathy”. The present review introduces the characteristics and findings of non-criteria manifestations observed in juvenile APS.

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