Levels Of Antiphospholipid Antibodies Research Articles

Antiphospholipid Patients Admitted in the Intensive Care Unit: What Must The Rheumatologist Know?

Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ failure. This review aims to offer insight and recent evidence on critically-ill APS patients. The CAPS classification criteria define this condition as the involvement of at least three organs/systems/tissues within less than a week, caused by small vessel thrombosis, in patients with elevated antiphospholipid antibodies levels. These criteria do not encompass the full spectrum of critically-ill thrombotic APS patients and they need to be cautiously used for the bedside diagnosis of CAPS. Thrombocytopenia is the laboratory hallmark of CAPS, sometimes dropping below 20G/L, but a complete thrombotic microangiopathy pattern is infrequent. Anticoagulation is the pivotal treatment for APS and CAPS, associated with improved outcome. Triple therapy - the combination of anticoagulation, high-dose corticosteroids, and either plasma exchange or intravenous immunoglobulins - remains the standard treatment for CAPS patients. Eculizumab, an anti-C5 monoclonal antibody, may be useful in refractory patients. Despite significant progress, CAPS mortality rate remains high. Its diagnosis and management are complex, requiring a close multidisciplinary cross talk between APS specialists and intensivists.

Vitamin D Status in Patients with Primary Antiphospholipid Syndrome (PAPS): A Systematic Review and Meta-Analysis.

Primary antiphospholipid syndrome (PAPS) is a systemic autoimmune disorder, characterised by consistently high levels of antiphospholipid antibodies, thrombosis, and/or pregnancy morbidity. Due to various suspected causes, deficient or insufficient levels of vitamin D in the serum have been reported in patients with PAPS; however, the reports have been sporadic and inconclusive. This systematic review and meta-analysis aimed to comprehensively evaluate the serum vitamin D levels in patients with PAPS compared to controls. A protocol was registered in PROSPERO (Registration No. CRD42019132128) and a systematic literature search was conducted through Google Scholar, PubMed, Web of Science, Scopus, and ScienceDirect databases without restricting language and year. Pooled prevalence, mean difference (MD), and odds ratio (OR) along with 95% confidence intervals (CI) were determined by using a random effects model. Study quality was assessed by the Joana Brigg's Institute (JBI) protocol and publication bias was evaluated by a trim and fill funnel plot, Begg's, and Egger's tests. The pooled prevalence of vitamin D deficiency and insufficiency was found to be 32.2% [95% CI: 16.3-48.2] and 61.5% [95% CI: 40.2-82.8], respectively. Serum levels of vitamin D were considerably lower in the PAPS patients compared to controls (MD: -5.75, 95% CI: -9.73 to -1.77; p = 0.005). Multiple sensitivity analyses showed that the results remained statistically significant, demonstrating the robustness of this meta-analysis. No significant publication bias was detected in determining the MD of serum vitamin D levels in PAPS and controls. In conclusion, PAPS patients had greater rates of vitamin D deficiency or insufficiency, higher frequency of thrombosis, and lower serum vitamin D levels than healthy individuals.

Antiphospholipid antibodies are enriched post-acute COVID-19 but do not modulate the thrombotic risk

Background and objectivesCOVID-19-associated coagulopathy, shown to increase the risk for the occurrence of thromboses and microthromboses, displays phenotypic features of the antiphospholipid syndrome (APS), a prototype antibody-mediated autoimmune disease. Several groups have reported elevated levels of criteria and non-criteria antiphospholipid antibodies (aPL), assumed to cause APS, during acute or post-acute COVID-19. However, disease heterogeneity of COVID-19 is accompanied by heterogeneity in molecular signatures, including aberrant cytokine profiles and an increased occurrence of autoantibodies. Moreover, little is known about the association between autoantibodies and the clinical events. Here, we first aim to characterise the antiphospholipid antibody, anti-SARS-CoV-2 antibody, and the cytokine profiles in a diverse collective of COVID-19 patients (disease severity: asymptomatic to intensive care), using vaccinated individuals and influenza patients as comparisons. We then aim to assess whether the presence of aPL in COVID-19 is associated with an increased incidence of thrombotic events in COVID-19. Methods and resultsWe conducted anti-SARS-CoV-2 IgG and IgA microELISA and IgG, IgA, and IgM antiphospholipid line immunoassay (LIA) against 10 criteria and non-criteria antigens in 155 plasma samples of 124 individuals, and we measured 16 cytokines and chemokines in 112 plasma samples. We additionally employed clinical and demographic parameters to conduct multivariable regression analyses within multiple paradigms. In line with recent results, we find that IgM autoantibodies against annexin V (AnV), β2-glycoprotein I (β2GPI), and prothrombin (PT) are enriched upon infection with SARS-CoV-2. There was no evidence for seroconversion from IgM to IgG or IgA. PT, β2GPI, and AnV IgM as well as cardiolipin (CL) IgG antiphospholipid levels were significantly elevated in the COVID-19 but not in the influenza or control groups. They were associated predominantly with the strength of the anti-SARS-CoV-2 antibody titres and the major correlate for thromboses was SARS-CoV-2 disease severity. ConclusionWhile we have recapitulated previous findings, we conclude that the presence of the aPL, most notably PT, β2GPI, AnV IgM, and CL IgG in COVID-19 are not associated with a higher incidence of thrombotic events.

Comparison of Antiphospholipid Antibody Levels in Children with and Without COVID-19

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has led to the recent pandemic. According to published reports, respiratory symptoms, such as pneumonia and inflammatory conditions, are common in this disease. Objectives: The current study aimed to investigate the level of antiphospholipid (aPL) antibodies in children with and without coronavirus disease 2019 (COVID-19). Methods: This descriptive-analytic cross-sectional study was conducted on patients under 16 years of age with and without COVID-19 admitted to Ali Asghar Hospital between December 2021 and February 2022. Patient information was collected by the researcher in a checklist. The checklist included demographic information, clinical findings, and information on laboratory and ultrasound results. Results: In this study, 99 patients were evaluated in three groups: control (without COVID-19), moderate, and severe. The means (standard deviation [SD]) of C-reactive protein (CRP) and D-dimer were significantly higher in the severe group. The Pearson correlation coefficient test was used to examine the relationship between aPL and anticardiolipin (aCL) antibodies with laboratory results. The only significant and direct relationship was observed between aCL antibody and D-dimer. Conclusions: Increased CRP and D-dimer in children with COVID-19 are associated with the severe form of this serious disease. However, there was no significant association between the severity of the disease and the levels of aCL and aPL antibodies and anti-beta 2-glycoprotein I antibodies (aβ2GPI) in children.

Effect of physiotherapeutic treatment on the quality of life in a patient with antiphospholipid syndrome: A case report

Antiphospholipid syndrome (APS) is a systemic autoimmune disease with elevated levels of antiphospholipid antibodies (aPL). The chronic course, multisystem involvement, limitation of physical activity and frequent fatigue lead to reduced health-related quality of life. There is insufficient scientific evidence in the literature regarding the feasibility and safety of using physical rehabilitation factors and their impact on functional activity and quality of life in these patients.

Hospitalized patients with positive antiphospholipid antibodies who have low complement levels are at increased risk for death—a retrospective cohort study

To investigate whether low complement levels can predict worse outcomes in patients hospitalized with positive anti-phospholipid antibodies. This was a retrospective cohort study. We obtained demographics, laboratory, and prognostic data of all consecutive patients hospitalized between 2007 and 2021, for whatever reason, with at least one positively abnormal anti-phospholipid antibody, who were also tested for complement levels (C3 or C4). We then compared the rates of long-term mortality, 1-year mortality, deep vein thrombosis, and pulmonary emboli between groups of low complement and normal complement levels. Multivariate analysis was used to control for levels of clinical and laboratory confounders. We identified 32,286 patients tested for anti-phospholipid antibodies. Of those patients, 6800 tested positive for at least one anti-phospholipid antibody and had a documented complement level. Significant higher mortality rates were found in the low complement group, with an odds ratio for mortality (OR 1.93 CI 1.63-2.27 p < .001). Deep vein thrombosis and pulmonary emboli rates were similar. Multivariate analysis confirmed that low complement was an independent predictor for mortality after controlling for age, sex, dyslipidemia, chronic heart failure (CHF), chronic kidney disease (CKD), and anemia. Our study results indicate that low complement is associated with significantly higher mortality rates in admitted patients with elevated levels of anti-phospholipid antibodies. This finding correlates with recent literature suggesting a vital role for complement activation in anti-phospholipid syndrome.

Changes in the Level of Antiphospholipid Antibodies (Anticardiolipin and Anti-β2-Glycoprotein-I) and Thromboembolic Indices in COVID-19 Patients during 3 Weeks.

COVID-19 is a respiratory disease caused by infection with severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2). Thrombotic complications appear to be of particular importance in patients with COVID-19. This study aimed to investigate Changes in the level of Antiphospholipid antibodies (Anticardiolipin and Anti-β2-glycoprotein-I) and thromboembolic indices in COVID-19 patients during 3 weeks. This cross-sectional study was performed on adults with Covid-19 hospitalized at Al-Zahra Hospital in Isfahan. The case group includes the patients admitted to the internal ward or ICU who despite receiving prophylactic or anticoagulant doses suffer from thrombotic complications and the control group includes COVID-19 patients without thromboembolic events. The sample size of 120 people was considered. Anticardiolipin and anti-β2-glycoprotein-I antibodies, coagulation profiles including Fibrinogen, PTT, PT Troponin, ESR, CRP, and D-dimer were examined. After collection, the data were entered into spss24 software and analyzed. The results showed that there was no statistically significant difference in the changes of anticardiolipin and anti-beta-2 glycoprotein in IgM and IgG as well as in the changes of ESR, CRP, PTT, PT, and fibrinogen in the two groups (P > 0.05). Our study showed that there was no statistically significant relationship between anti-phospholipid antibodies (anticardiolipin and anti-beta-2 glycoprotein) and thromboembolic events. Therefore anticardiolipin and anti-beta-2 glycoprotein is probably the puzzles causing thrombosis in COVID-19 patients, and other inflammatory responses should be examined among the cases.

Antiphospholipid antibodies as biomarkers for schizophrenia

Background: Schizophrenia is a neurodegenerative psychiatric disorder causing substantial personal and societal burden, characterized by positive, negative and disorganized symptoms and it is also associated with cognitive impairment. Complex interactions of genetic and environmental factors have been implicated in etiology of schizophrenia. Diagnosis is a challenging problem due to the heterogeneity of this mental disorder and lack of specific effective biomarkers. The antiphospholipid antibodies may have a causal role in the development of some neuropsychiatric conditions such as psychosis, depression and dementia.Aims: To study the relationship between antiphospholipid antibodies and schizophrenia. In addition, the relationship between the level of these antibodies and psychopathological symptoms were evaluated.Patients and Methods: Thirty schizophrenic patients compared with 30 healthy people were included in this study. Psychiatric symptoms severity, global cognitive functions and serum levels of antiphospholipid antibodies were evaluated twice in schizophrenic patients with interval one and half month under treatment and compared to the healthy control results.Results: There were statistically significant differences between case and control groups regarding the studied cognitive domains and antiphospholipid IGM. In the case group, there were a direct correlation between antiphospholipid IGM and positive schizophrenic symptoms, and inverse correlation between cognitive scales and negative symptoms.Conclusion: The increased levels of antiphospholipid IGM antibodies were correlated with positive schizophrenic symptoms while negative symptoms were correlated with cognitive scales among case group. These results may give an important implication for the usefulness of antiphospholipid antibodies screening in treating schizophrenia in the future.

A Comparative Analysis of Anticardiolipin, Anti-Β2-Glycoprotein-1, and Lupus Anticoagulants in Saudi Women with Recurrent Spontaneous Abortions.

Association and pathogenesis of antiphospholipid antibodies (APAs) in patients with Antiphospholipid syndrome (APS) as well as systemic lupus erythematosus (SLE) suffering from unexplained spontaneous abortions is controversial. Moreover, the majority of the published evidence is based on the patient histories rather than the study groups. The main objective of the present study is to do a comparative analysis of the high levels of antiphospholipid antibodies (APAs) including anticardiolipin (ACA), anti-β2-glycoprotein-1 (β2 GP1), and Lupus anticoagulants (LA) and their association with the gestational age of abortion and duration past abortion in Saudi women. In this study, 100 women living in Makkah city, located in the Western region of Saudi Arabia, with a mean age of 30.5 ± 12.60 years (mean ± standard deviation), having a previous history of recurrent spontaneous abortions were enrolled as case group and 100 healthy pregnant women previously having one or more successful pregnancies were selected as control group. Based on the gestational age of the patient’s miscarriages, our results revealed consistently and significantly high levels of ACA, β2 GP1, and LA, being greatest at more than 15 weeks when compared with 10 weeks and 11−15 weeks, respectively. Based on duration of past miscarriages, a slightly increased level was observed at ≤4 years after their first miscarriage. In addition, less or more equal levels of these antibodies were observed at 5−12 and more than 12 years in all the cases of miscarriages. We concluded an elevated pattern of APAs in these patients with an increase in the age of abortion. A comparison between the levels of ACA, β2 GP1, and LA further indicated a highly significant level of LA in all the cases of abortions (p < 0.0005).

Painful unilateral facial swelling due to superficial temporal artery thrombosis: a rare presentation of Antiphospholipid Syndrome

Introduction Persistent painful unilateral temporal swelling is rarely seen in clinical practice. Antiphospholipid syndrome (APS) is an autoimmune and systemic disorder that causes changes in blood clotting homeostasis, marked by arterial or venous thrombosis, gestational morbidity, and high and persistently positive serum levels of antiphospholipid antibodies (aPL). APS is more common in young women and middle-aged adults, with no preference for race. Among its clinical features, temporal artery thrombosis, associated with headache and temporal and hemifacial edema is extremely rare, with few publications worldwide on this topic. Objective This report aims to present the case of a unilateral painful facial edema due to thrombosis of the superficial temporal artery as an unprecedented manifestation of Antiphospholipid Syndrome. Thus, the only record in the English-language literature surveyed in the Pubmed database in July 2022 highlights the rarity of this APS presentation and the consequent challenge in suspecting the correct diagnosis for adequate treatment. Methods Case report. Data disclosure was authorized by the patient through an informed consent form. Results A 32-year-old woman was presented with pain in the right temporal region of her face. The pain was intense, daily and continuous, pulsating, without irradiation, which worsened with physical activity and presented partial relief with common analgesics. After 20 days, she developed a right pale temporal edema (Figure 1) associated with a significant worsening of pain and intense right unilateral headache attacks triggered by chewing and speaking. She has a history of deep vein thrombosis in the left lower limb. Physical examination and imaging tests showed significant cold edema of the right temporal region with asymmetry of the temporal muscles, which was extremely painful on palpation and made it difficult to open the mouth. There were no other changes in the general physical... (Too see the complete abstract, please, check out the PDF.)

Adaptive immune responses in patients requiring revision after total knee arthroplasty.

Dissatisfaction occurs in nearly 20% of patients after total knee arthroplasty (TKA); however, there remains only limited understanding of the biologic mechanisms that may contribute to suboptimal postoperative outcomes requiring revision surgery. Expansion of effector T and B cells, could promote an abnormal healing response via local or peripheral immune system mechanisms and contribute to inferior outcomes necessitating revision TKA. In this pilot study, we hypothesized that patients suffering from complications of arthrofibrosis or instability may exhibit differences in adaptive immune function. Patients (n = 31) undergoing revision TKA for an indication of arthrofibrosis or instability were prospectively enrolled. Whole blood and synovial fluid (SF) from the operative knee were collected at time of surgery. Peripheral blood mononuclear cells were isolated and analyzed by flow cytometry. Serum and SF were assessed for immunoglobulin levels by Luminex and antiphospholipid antibodies by enzyme-linked immunoassay. No significant differences were observed in peripheral blood T/B cell populations or serum immunoglobulins levels between groups. SF analysis demonstrated significant differences between the two groups, with higher levels of immunoglobulin G1 (IgG1) (p = 0.0184), IgG3 (p = 0.0084) and antiphosphatidyl serine IgG (p = 0.034) in arthrofibrosis relative to instability patients. Increased levels of both IgG subclasses and antiphospholipid antibodies in the SF suggest that intra-articular T-B cell interactions, potentially triggered by exposure to apoptotic components generated during post-op healing, could be functioning as a source of immune complexes that fuel fibrous tissue growth in arthrofibrotic patients.

OA04 Paediatric APS - children are not just small adults

OA04 Paediatric APS - children are not just small adults

Anti-Phosphatidylethanolamine and Anti-Phosphatidylserine Antibodies-Association with Renal Involvement, Atherosclerosis, Cardiovascular Manifestations, Raynaud Phenomenon and Disease Activity in Polish Patients with Systemic Lupus Erythematosus.

Objective. To evaluate the association between anti-phosphatidylethanolamine (aPE) and anti-phosphatidylserine (aPS) antibodies and cardiovascular risk, organ involvement and disease activity in systemic lupus erythematosus (SLE) patients. Methods. We studied 93 SLE patients and 30 controls. We analyzed levels of anti-phospholipid antibodies, including aPS and aPE, the profiles of antinuclear, anti-neutrophil cytoplasmic (ANCA) and anti-endothelial antibodies, carotid intima-media thickness (cITM) and atherosclerotic plaque presence, ankle-brachial and high resistance indices, atherosclerotic risk factors, organ manifestations and treatment. Results. Levels of aPS and aPE were significantly higher in SLE patients in comparison with the controls (p = 0.038 and p = 0.044, respectively). aPS was associated with the risk of Raynaud’s phenomenon (p = 0.021) development. aPE increased the risk of renal involvement (p = 0.049), cerebral stroke (p = 0.050), high vlues of cIMT (p = 0.041) development as well as occurrence of selected serological markers associated with activity of the disease such as anti-double stranded DNA (p = 0.021). The long duration of regular smoking (p = 0.021) and the high number of cigarettes/day (p = 0.015) were significantly associated with the risk of aPE occurrence. Conclusions. Patients with aPS and aPE are at risk of vascular involvement. Especially the presence of aPE may significantly increase the risk of thrombotic complications development in SLE patients without classical serological markers of APS. Finally, aPE might be used as a marker of disease activity and risk of renal injury development in this patient group. The classical atherosclerotic markers including lipid indices play an important role in complex analysis of cardiovascular risk in lupus patients and enable to identify patients at the highest risk and implement effective preventive, diagnostic and therapeutic procedures.

Immunomodulatory Effects of Bio-Clean II Herbal Remedy on C-reactive protein, Corticosterone and Antiphospholipid antibodies in Rats exposed to Purified Bacterial Lipopolysaccharide

Background: Bio-Clean II has been previously shown to fight viral infection, boost immunity, and possesses anti-inflammatory properties by regulating the serum level of inflammatory cytokines, as well as T-Helper 4 and Cytotoxic T-Lymphocytes in rats exposed to purified bacterial lipopolysaccharide (LPS). Aim: The aim of this study is to assess the effects of Bio-Clean II on the C-reactive protein (CRP), corticosterone (CORT) and anti-phospholipid antibodies (aPLs) levels in rats exposed to bacterial lipopolysaccharide (LPS) using animal model. Materials and Methods: A total of 36 male Wistar rats weighing 150g±50g (mean±SD) were purchased and randomly assigned to six (6) groups of 6 rats each. Group 1, 2, 3, 5 and 6 were induced with a single dose of 5mg/Kg of purified LPS® (E.coli 0127:B8, Sigma-Aldrich, St. Louis, USA), administered through intraperitoneal route using 1ml sterile needle and syringe, except for group 4 which served as the zero control (given water and food only throughout the experiment). Group 1 served as the inflammation control. Group 2 which served as the positive control received 50 mg diclofenac/kg [bid] and 500 mg ciprofloxacin/kg [bid] (positive control) in place of the Bio-Clean. Group 3 which served as the negative control received sterile phosphate buffer saline (PBS). While rats in group 5 and group 6 were treated orally with the herbal remedy “Bio-Clean II” for 7 days and 14 days, respectively. After which, the rats were killed and a cardiac blood specimen was taken from each rat and transferred to plain bottles to clot. Serum was obtained from the clotted blood by centrifugation. The serum levels of C-reactive protein, corticosterone and antiphospholipid antibodies were assayed using ELISA kits, supplied by Elabscience Biotechnology Inc, USA. Data generated were subjected to Statistical Package for Social Scientists-Version 20 (SPSS-20). Results: The outcome of this results show that the serum level of C-reactive protein of the 7 days (1.05±0.06ng/ml) and 14 days (0.93±0.05ng/ml) Bio-Clean II treated rats was found to be significantly lower (p=0.002 and p=0.000, respectively) when compared to the inflammation control group (1.70±0.07ng/ml). Similarly, the serum level of anti-phospholipid antibodies of the 7 days (6.40±0.67 Uml) and 14 days (4.27±0.66 U/ml) Bio-Clean II treated rats was found to be significantly lower (p=0.02 and p=0.008, respectively) when compared to the inflammation control group (16.47±1.53 Uml). Meanwhile, the corticosterone level of the 7 days Bio-clean treated rats (9.40±1.30ng/mL) was found to be non-significantly lower (p=0.812) in comparison to the inflammation control (13.50±2.50ng/mL); while that of the 14 days Bio-clean II treated rats (6.80±1.00ng/mL) was significantly lower (p=0.026). Conclusion: The outcome of this study underscores the anti-inflammatory potential of Bio-Clean II in the treatment of bacterial inflammatory diseases.

The effect of the BNT162b2 vaccine on antinuclear antibody and antiphospholipid antibody levels.

The Food and Drug Administration (FDA) approved the first SARS-CoV-2 mRNA vaccine (Pfizer-BioNTech) in December 2020. New adverse events have emerged since these vaccines have reached market. Although no clear association between messenger ribonucleic acid (mRNA) vaccines and autoimmunity has emerged, the significance of such an association warrants further exploration. After obtaining consent, a standardized survey on baseline characteristics and other relevant variables was conducted on unvaccinated individuals who were scheduled for vaccination and had not previously contracted COVID-19. Blood samples were collected from participants prior to the first dose, prior to the second dose, and 1 month after the second dose. All collected samples were tested for antinuclear antibody (ANA) titers using indirect immunofluorescence microscopy kits, and antiphospholipid (APS) immunoglobulin M (IgM) and immunoglobulin G (IgG) levels using an enzyme-linked immunoassay (ELISA) technique. ANA titers were positive for 9 participants out of 101 (8.9%) in the first pre-vaccination draw. For the second draw, the number of participants testing positive for ANA decreased to 5 (5%). For the last draw, 6 (5.9%) participants tested positive for ANA titers. One participant tested positive for APS IgM at the first pre-vaccination draw, 2 tested positive at the second draw, and 2 at the third draw. As for APS IgG titers, all participants tested negative in the three draws. McNemar’s test for two dependent categorical outcomes was conducted on all variables and did not show a statistical significance. The McNemar test of these two composite variables (i.e., ANA/APS, first draw vs. ANA/APS, second and third draws) did not show statistical significance. The 2-sided exact significance of the McNemar test was 1.0. The Friedman test also showed no significance (p = 0.459). No association was found between BNT162b2 vaccine administration and changes in APS and ANA titers. The benefits of the BNT162b2 vaccine significantly outweigh any possible risk of autoimmune dysregulation considering the current evidence.

Determination of Thrombogenicity Levels of Various Antiphospholipid Antibodies by a Modified Thrombin Generation Assay in Patients with Suspected Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is a hypercoagulable state accompanied by the presence of heterogeneous antiphospholipid antibodies (aPL), which nonspecifically affect hemostasis by the presence of lupus anticoagulans (LA), anticardiolipin antibodies (aCL), antibodies against β2-glycoprotein-I (anti-β2GPI), but also non-criteria antibodies such as antibodies against β2-glycoprotein-I domain I (anti-DI), anti-phosphatidylserine/prothrombin (anti-PS/PT), anti-annexin V, and many others. The main target of the antibodies is the activated protein C (APC) system, the elimination of which can manifest itself as a thrombotic complication. The aim of this study was to determine the thrombogenicity of antibodies using a modified protein C-activated thrombin generation assay (TGA) on a group of 175 samples suspected of APS. TGA was measured with/without APC and the ratio of both measurements was evaluated (as for APC resistance), where a cut-off was calculated ≤4.5 (90th percentile) using 21 patients with heterozygous factor V Leiden mutation (FV Leiden heterozygous). Our study demonstrates the well-known fact that multiple positivity of different aPLs is a more severe risk for thrombosis than single positivity. Of the single antibody positivity, LA antibodies are the most serious (p value < 0.01), followed by aCL and their subgroup anti-DI (p value < 0.05). Non-criteria antibodies anti-annexin V and anti-PT/PS has a similar frequency occurrence of thrombogenicity as LA antibodies but without statistical significance or anti-β2GPI1 positivity. The modified TGA test can help us identify patients in all groups who are also at risk for recurrent thrombotic and pregnancy complications; thus, long-term prophylactic treatment is appropriate. For this reason, it is proving increasingly beneficial to include the determination antibodies in combination with modified TGA test.

Autoantibodies and SARS-CoV-2 infection: A case report

The article describes a COVID-19 patient with several biomarkers of systemic cytokine storm (including multiple excess over the reference values of C-reactive protein, ferritin and D-dimer) and increased level of autoantibodies (aPL and anti-CCP). At the same time contrast-enhanced CT and US failed to detect pulmonary embolism or lower limb deep vein thrombosis. Three months after discharge from the hospital, a high level of antiphospholipid antibodies and D-dimer remained. The relationship between infection with SARS-CoV-2 and autoimmunity has been discussed.

Simultaneous Onset of Pediatric Systemic Lupus Erythematosus in Twin Brothers: Case Report

There are hundreds of twin adult patients with systemic lupus erythematosus (SLE), but male children with SLE are rarely affected. Two monozygotic twin brothers developed SLE at the age of 11 years during 1 month. The index brother manifested with Henoch-Shonlein purpura, accompanied by ANA positivity, and later developed critical left femoral arterial stenosis with high levels of anti-dsDNA, antiphospholipid antibodies, hypocomplementemia, and Coombs-positive hemolytic anemia. At that time his twin brother had only identical autoimmune findings and developed clinical manifestation (myositis and fasciitis) a month later. Both twins had increased IFN-score and shared a heterozygous variant in the RNASEL gene. Index patients developed scalp rash and nephritis 6 months after their parents refused the treatment which has been lasted for 1 year after disease diagnostics.ConclusionThe simultaneous onset of the pediatric SLE in the male twin is a very rare situation suspected monogenic origin of the disease. Further functional studies are required to confirm the causative role of the mutation.

Abstract 461: Antiphospholipid Antibodies In Covid-19 Convalescent Plasma

Background: Although convalescent plasma has historically yielded significant benefits in the treatment of infectious diseases, numerous clinical trials have not shown a consistent benefit in treating either moderately or severely ill COVID-19 patients with plasma from convalescent patients. We previously reported increased levels of antiphospholipid antibodies (aPL) in acutely ill COVID patients, with high aPL titers correlating with more neutrophil extracellular traps (NETs) and poorer patient outcomes. We hypothesized that persistence of potentially deleterious autoantibodies in previously hospitalized, convalescent plasma donors may activate immune cells and add context for the lack of clinical efficacy in COVID. Methods: 116 samples of convalescent plasma from donors previously hospitalized with COVID were obtained from Vitalant. Antibody titers (IgG, IgM, IgA) of anti-2-glycoprotein 1 (a2GP1) and anti-cardiolipin (aCL) and anti-phosphatidylserine/prothrombin (IgG, IgM) were quantified. NET formation by healthy human neutrophils treated with convalescent plasma was evaluated in vitro. Results: High aPL titers &gt;40 U/mL) were observed in 10/116 (8.6%) convalescent samples, and 6/10 were positive for either IgG or IgM aCL. The aPL positivity rate exceeded the expected background population rate of ~3%. Six of 116 convalescent samples increased NET-associated MPO activity in purified, primary neutrophils from healthy donors compared with control plasma (mean increase of 23% +/- 11, p&lt;0.01), where only 2 of these were aPL-positive samples. Conclusion: aPL were elevated in a larger proportion of surveyed convalescent plasma samples than reported in the healthy population. aPL positivity did not correlate with purified neutrophil activation, suggesting aPL in donor plasma does not have a direct, activating effect on healthy neutrophils. Considering the complex interactions among circulating blood cells, ongoing experiments are examining the impact of convalescent plasma on platelet and endothelial cell function to further elucidate potential autoantibody-mediated vascular perturbations. The results of these studies will be important in informing the vascular implications and safety of convalescent plasma in COVID.

A case of primary antiphospholipid antibody syndrome with intermittent cerebral venous sinus thrombosis manifested with recurrent epilepsy

Antiphospholipid syndrome (APS) is an acquired autoimmune clinical disorder characterized by persistently raised levels of anti-phospholipid antibodies and clinical feature of hypercoagulability (recurrent vascular thrombosis and pregnancy morbidities). Primary APS is idiopathic in nature though secondary APS is associated with other connective tissue diseases like SLE. Here, we present an instance of primary APS with recurrent cerebral venous sinus thrombosis in a youthful unmarried female who clinically presented with generalized epilepsy. Level of anti-cardiolipin (aCL) and anti-beta 2 GP1 antibodies were found elevated on two occasions 12 weeks apart. The patient was treated with anticoagulation therapy along with anti-epileptic therapy and she responded well to the therapy. This case report highlights that clinicians should always suspect APS in female patients who had history of seizures and ischemic or thrombotic lesions on brain imaging, irrespective of presence of pregnancy comorbidities. Keywords: antiphospholipid syndrome; cerebral venous; sinus thrombosis; epilepsy