scholarly journals La sindrome da anticorpi anti-fosfolipidi: un mare di autoanticorpi

2021 ◽  
Vol 24 (6) ◽  
pp. 187-187
Author(s):  
Eleonora Dei Rossi
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
High Titer ◽  
Small Vessel ◽  
Pulmonary Thrombosis ◽  
Systemic Disorder ◽  
Vessel Thrombosis

Antiphospholipid syndrome is an autoimmune systemic disorder characterized by arterial, venous or small vessel thrombosis in the setting of documented persistent antiphospholipid antibodies that include the lupus anticoagulant or moderate-high titer anticardiolipin or anti-β2Glycoprotein I antibodies. The paper describes a case of a 16-year-old girl with recurrent pulmonary thrombosis and antiphospolipid syndrome.

The clinical relevance of isolated lupus anticoagulant positivity in patients with thrombotic antiphospholipid syndrome

2020 ◽  
Author(s):  
Dong-mei Yin ◽  
Philip de Groot ◽  
Marisa Ninivaggi ◽  
Katrien M.J. Devreese ◽  
Bas de Laat
Keyword(s):  
Antiphospholipid Syndrome ◽  
Clinical Relevance ◽  
Antiphospholipid Antibodies ◽  
Odds Ratio ◽  
Lupus Anticoagulant ◽  
Solid Phase ◽  
Control Group ◽  
Domain I ◽  
Normal Controls ◽  
Better Than

Background: Patients positive for three types of antiphospholipid antibodies (aPLs) (triple positivity) have been identified at a high risk for thrombotic events. However, the clinical significance of isolated lupus anticoagulant (LAC) positivity is debated. Objectives: To investigate the clinical relevance of isolated LAC. Patients/Methods 456 patients were enrolled in this study; 66 antiphospholipid syndrome patients and 390 control patients. The control group existed of autoimmune patients (n=91), patients with thrombosis but without aPLs (n=127) and normal controls (n=172). The criteria LAC, anti-cardiolipin (anti-CL) and anti-beta2glycoprotein I (anti-β2GPI) IgG and IgM and the non-criteria IgA anti-CL and anti-β2GPI, anti-domain I (anti-DI) of β2GPI IgG and anti-phosphatidylserine/prothrombin (anti-PS/PT) IgG and IgM were detected according to the ISTH guidelines for solid phase assays. Results: 70 patients were positive for LAC, of which 44 were negative for both anti-β2GPI and anti-CL. We found that isolated LAC proved to be strongly associated with vascular thrombosis (Odds ratio (OR) (95% CI) 7.3 (3.3-16.1)), even better than triple positive samples (OR 4.3 (1.6-12.2)). The titers of the anti-PS/PT IgG and IgM were significantly higher in triple positivity samples compared to samples with isolated LAC positivity. The majority of single LAC positives were anti-PS/PT negative. We observed that LAC positivity was weaker in isolated LAC positive patients compared to LAC activity in triple positive patients. Conclusions: Isolated LAC was highly associated with thrombosis. The presence of anti-PS/PT could not explain LAC positivity in isolated LAC. Isolated LAC showed a weaker LAC activity compared to triple positive patients.

Stillbirth: the impact of antiphospholipid syndrome?

Lupus ◽  
2016 ◽  
Vol 26 (3) ◽  
pp. 237-239 ◽  
Author(s):  
C A Herrera ◽  
C C Heuser ◽  
D Ware Branch
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Low Dose ◽  
Fetal Death ◽  
Therapeutic Approaches ◽  
New Therapeutic Approaches ◽  
Dose Aspirin ◽  
Low Dose Aspirin ◽  
The Impact

Fetal death resulting in stillbirth is generally acknowledged as a feature of antiphospholipid syndrome. Recently published studies appear to confirm the association between antiphospholipid antibodies (aPL) and stillbirth, though additional studies of better design would be welcome. Emerging evidence suggests that treatment with heparin agents and low dose aspirin to prevent fetal death is imperfect. New therapeutic approaches for patients with lupus anticoagulant or triple aPL positivity are needed.

scholarly journals Loss of antiphospholipid antibody positivity post-thrombosis in SLE

2020 ◽  
Vol 7 (1) ◽  
pp. e000423
Author(s):  
Muznay Khawaja ◽  
Laurence Magder ◽  
Daniel Goldman ◽  
Michelle A Petri
Keyword(s):  
Clinical Practice ◽  
Prospective Study ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Complete Loss ◽  
Antiphospholipid Antibody ◽  
Confirmatory Tests ◽  
Antibody Positivity

Background/PurposeLoss of positivity of antiphospholipid antibodies has been observed in clinical practice post-thrombosis in patients with SLE with secondary antiphospholipid syndrome (APS). Our study defined the frequency of this loss and the duration before positivity recurred.MethodsIn this prospective study, patients with SLE having at least two positive antiphospholipid markers prior to thrombosis and at least 1 year of follow-up after thrombosis were included. Antiphospholipid markers included lupus anticoagulant (dilute Russell viper venom test >45 s followed by mixing and confirmatory tests) and/or anticardiolipin titre (aCL IgG ≥20, aCL IgM ≥20 and/or aCL IgA ≥20). The percentage of visits with positive antiphospholipid markers after thrombosis was calculated. For patients with a negative antiphospholipid marker any time after thrombosis, survival estimates were performed to calculate the time to return of antiphospholipid positivity.ResultsIn APS due to SLE, complete loss of antiphospholipid positivity post-thrombosis was up to 41% for aCL IgG, 51% for IgM and 50% for IgA, but only 20% for those with lupus anticoagulant. Of those who at some point lost aCL IgG or became negative for lupus anticoagulant, the majority (60% and 76%, respectively) reacquired the antibody within 5 years. In contrast, of those who lost aCL IgM or IgA, fewer reacquired it within 5 years (37% and 17%, respectively).ConclusionIntermittent positivity of antiphospholipid antibodies is present in APS due to SLE. These fluctuations make it difficult to decide on length of anticoagulation. Lupus anticoagulant is more likely to persist post-thrombosis.

The role of antiphospholipid antibodies in reproductive failure

1997 ◽  
Vol 6 (3) ◽  
pp. 133-143
Author(s):  
D Ware Branch ◽  
Harry H Hatasaka
Keyword(s):  
Pregnancy Outcome ◽  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Fetal Loss ◽  
Placental Insufficiency ◽  
Antiphospholipid Antibody ◽  
The Relationship

The relationship between antiphospholipid antibodies and the clinical features of placental insufficiency, pre-eclampsia, and fetal loss has emerged as one of the most exciting new observations in obstetrics in the last 15 years. Antiphospholipid syndrome is the only convincing ‘immunologic’ disturbance of pregnancy affecting the fetus other than anti-erythrocyte or antiplatelet alloimmunization disorders, and it is now routine to test patients with fetal loss for the two best characterized antiphospholipid antibodies, lupus anticoagulant and anticardiolipin. Although there is no proven mechanism for fetal loss, treatment of antiphospholipid antibody-positive mothers during pregnancy with heparin improves pregnancy outcome.

scholarly journals Antiphospholipid syndrome accompanying systemic lupus erythematosus

2002 ◽  
Vol 55 (3-4) ◽  
pp. 89-96 ◽  
Author(s):  
Gorana Mitic
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Clinical Manifestations ◽  
Spontaneous Abortions ◽  
Systemic Lupus ◽  
Recurrent Spontaneous Abortions ◽  
Thrombotic Complications

The aim of the study was the assessment of the prevalence of antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE). 72 patients with SLE had been investigated, 66 females and six males, aged 17 to 70 years, average 37,03. The presence of APA was determined using both ELISA assay for antiphospholipid antibodies ASSERACHROM APA by Diagnostica Stago and clotting tests for lupus anticoagulant: activated partial thromboplastin time (aPTT), tissue thromboplastin inhibition test (TTI) and dilute Russell viper venom time (dRVVT). Antiphospholipid antibodies have been found in 24 patients (33.44%), 10 of them were. with positive lupus anticoagulant tests, 6 of them were with positive ELISA test, while 8 of them had positive coagulation and immunological tests. Clinical manifestations that could be related to antiphospholipid syndrome were present in 22 patients (30.5%). The most common were thrombotic complications in 16 patients (22.25), recurrent spontaneous abortions in 7 patients (9.7%) and thrombocytopenia in 1 patient (1.39%). Presence of antiphospholipid syndrome was determined in 15 patients (20.83%). We can conclude that there is a significant correlation between presence of antiphospholipid antibodies and both thrombotic events and recurrent spontaneous abortions in SLE patients. Occurrence of thrombotic complications is in direct correlation with the level of antiphospholipid antibodies.

High-Dose Intravenous Immunoglobulin Treatment of a Pregnant Patient with an Antiphospholipid Syndrome: Immunological Changes Associated with a Successful Outcome

1994 ◽  
Vol 71 (06) ◽  
pp. 741-747 ◽  
Author(s):  
J Arnout ◽  
B Spitz ◽  
C Wittevrongel ◽  
M Vanrusselt ◽  
A Van Assche ◽  
...  
Keyword(s):  
Platelet Count ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Interaction Analysis ◽  
High Titer ◽  
Anticardiolipin Antibodies ◽  
Successful Outcome ◽  
High Dose ◽  
Slight Reduction

SummaryA patient with a history of habitual abortion, deep venous thrombosis, thrombocytopenia, high titer IgG anticardiolipin antibodies and a clearly positive lupus anticoagulant, was treated during her seventh pregnancy with high-dose intravenous immunoglobulins (IVIg) from the third month onwards. Every month, a daily infusion of 400 mg immunoglobulins per kg body weight was given during five consecutive days. The patient’s pregnancy ended preterm with a live birth, delivered by caesarian section because of a placental abruption. The 1070 g (P20-P25) weighing girl was in good health, apart from a bradycardia, due to dysfunction of the atrioventricular conduction.Each treatment with IVIg resulted in a slight reduction of both anticardiolipin antibodies and lupus anticoagulant levels and in an increase in platelet count. During the six-month observation period, a gradual decline in antiphospholipid antibodies and an increase in platelet count was found. The potential role of anti-idiotypic antibodies, present in the IVIg used for treatment, was studied. In vitro, IVIg were able to reduce the binding of the patient’s anticardiolipin antibodies to cardiolipin coated microtiter plates. The presence of anti-idiotypic antibodies in IVIg was further documented by affinity chromatography and by realtime biospecific interaction analysis (BIA) on a BIA-core instrument. Affinity purified anticardiolipin antibodies were retarded on a column of insolubilized IVIg and a weak interaction was found between IVIg and affinity purified patient antiphospholipid antibodies, coupled to the BIA-core biosensor. In addition, the same technology revealed increased levels of anti-antiphospholipid antibodies in the patient’s plasma following IVIg therapy. The partial and transient reduction of anti-phospholipid antibody levels observed immediately following each treatment course seems compatible with low affinity complexation of idiotype-antiidiotypes, resulting in an accelerated clearance of the autoantibodies. Despite the low affinity for the patient’s idiotypes, the beneficial long term effects observed could be related to an immune regulatory role of these anti-idiotypic antibodies on the synthesis of antiphospholipid antibodies.

Antiphosphatidylethanolamine antibodies and the antiphospholipid syndrome

Lupus ◽  
2009 ◽  
Vol 18 (10) ◽  
pp. 920-923 ◽  
Author(s):  
M Sanmarco ◽  
M-C Boffa
Keyword(s):  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Anticardiolipin Antibodies ◽  
Anionic Phospholipids ◽  
Glycoprotein I ◽  
Antiphosphatidylethanolamine Antibodies

The antiphospholipid antibodies included as laboratory criteria of the antiphospholipid syndrome (APS) are antibodies reacting with anionic phospholipids – anticardiolipin antibodies and lupus anticoagulant – and with β2-glycoprotein I. However, antibodies reacting with phosphatidylethanolamine (aPE), a zwitterionic phospholipid, have also been described to be associated with the main features of APS. The objectives of this review are to describe the characteristics of aPE and to bring attention to recent evidence that aPE are correlated with the main clinical features of APS, notably, in the absence of the laboratory criteria of this syndrome.

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

The Clinical Relevance of Noncriteria Antiphospholipid Antibodies

2017 ◽  
Vol 44 (05) ◽  
pp. 453-457 ◽  
Author(s):  
Giovanni Sanna ◽  
Maria Bertolaccini
Keyword(s):  
Antiphospholipid Syndrome ◽  
Clinical Relevance ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Clinical Utility ◽  
Diagnostic Value ◽  
Anticardiolipin Antibodies ◽  
Coagulation Cascade ◽  
Glycoprotein I

AbstractWhile lupus anticoagulant (LA), anticardiolipin antibodies (aCL), anti-β2 glycoprotein I (anti-β2GPI) antibodies represent the best available and the most widely used tests in the investigation for antiphospholipid syndrome (APS), evidence gathered in recent years indicates that other antiphospholipid antibodies (aPL) specificities may also play a role in the syndrome. Several autoantibodies have been shown to be complexed with phospholipids other than cardiolipin, or to some domains of β2GPI, or else directed to other proteins of the coagulation cascade, and these have also been proposed to be of relevance to APS, and their diagnostic value and clinical utility are the focus of current research.

scholarly journals Cerebrovascular events in patients with isolated anti-phosphatidyl-serine/prothrombin antibodies

2021 ◽  
Author(s):  
Massimo Radin ◽  
Alice Barinotti ◽  
Silvia Grazietta Foddai ◽  
Irene Cecchi ◽  
Elena Rubini ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Clinical Utility ◽  
Thrombin Generation ◽  
Phosphatidyl Serine ◽  
Positive Test ◽  
Thrombin Generation Assay ◽  
Cerebrovascular Events ◽  
History Of

AbstractThe interest of extra-criteria antiphospholipid antibodies is growing, especially in patients negative for conventional antibodies. In this study we aimed to assess the clinical utility of anti-phosphatidyl-serine/prothrombin antibodies (aPS/PT) testing in patients negative for Beta2-Glycoprotein 1(β2GPI)-dependent tests, for identifying antiphospholipid syndrome (APS) patients that developed cerebrovascular events (CVE). When screening APS patients attending our center, out of 119 aPS/PT IgG/IgM-positive patients, thus patients negative for aβ2GPI and aCL, 42 patients (35%) tested negative for β2GPI-dependent tests and were tested with thrombin generation assay (TGA). Ten patients (24%), with isolated aPS/PT IgG/IgM, had a history of CVE. Lupus anticoagulant (LA)-positive test was more frequently observed in patients with CVE (8/22 vs. 2/20; p = 0.045). Out of the 10 patients who experienced CVE, 3 patients were aPS/PT IgG positive (all LA positive), and 8 patients were aPS/PT IgM positive (6/8 LA positive). One patient was positive for both aPS/PT IgG and IgM. LA-positive patients had only high titers of aPS/PT IgG/IgM, all of them being ≥ 80 U/ml, while the 2 LA-negative patients were aPS/PT IgM positive with medium titers [40–60 U/ml]. LA-positive patients had significantly altered TGA profile when compared to those who were LA negative, considering all TGA parameters. LA-positive patients had significantly higher tLag (8.4 ± 3.3 min vs. 6.6 ± 1.8 min; p = 0.046), higher tPeak (14 ± 4.3 min vs. 11 ± 2.7 min; p = 0.015) and lower Peak (207 ± 152 nM vs. 356.3 ± 104.7 nM; p < 0.001) and lower AUC (2109.7 ± 1006.9 nM vs. 2772.5 ± 776.8 nM; p = 0.033). The use of aPS/PT might be of help in identifying patients with CVE and APS, as also confirmed by TGA testing.

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