Presence Of Antiphospholipid Antibodies Research Articles

Bilateral Adrenal Hemorrhage in a Patient Anticoagulated with Apixaban for Antiphospholipid Syndrome (APS): A Rare Case Report

Context: Adrenal hemorrhage is a recognized yet rare complication associated with antiphospholipid syndrome (APS), an autoimmune disorder characterized by the presence of antiphospholipid antibodies that increase the risk of thrombosis. The incidence of adrenal hemorrhage in patients with APS has a diagnostic yield of approximately one-third, indicating that it may often go unrecognized in clinical practice. Traditionally, the management of APS has relied on anti-vitamin K therapy, such as warfarin. However, the advent of direct oral anticoagulants (DOACs) has prompted discussions about their use as an alternative. Recent clinical guidelines, however, strongly advise against the use of DOACs in patients with APS due to concerns over their safety and efficacy in preventing thromboembolic events. Case Presentation: This case study describes a 53-year-old woman with a history of venous thromboembolism who was treated with apixaban, a direct oral anticoagulant. She presented with acute abdominal pain and symptoms suggestive of adrenal insufficiency. Initial evaluations revealed significant changes in her clinical condition, leading to further diagnostic imaging. A CT scan demonstrated bilateral adrenal hemorrhage, which was a surprising finding given her anticoagulation therapy. Subsequent laboratory tests indicated a positive triple antibody profile, confirming a diagnosis of primary antiphospholipid syndrome. This case is particularly noteworthy as there have been limited documented instances of adrenal hemorrhage in patients receiving chronic anticoagulation with apixaban. The presentation of bilateral adrenal hemorrhage in the context of APS adds a crucial dimension to the understanding of the disease and its management. Conclusion: This case highlights the potential for serious complications, such as adrenal hemorrhage, in patients with antiphospholipid syndrome, particularly those treated with direct oral anticoagulants. It underscores the necessity for clinicians to remain vigilant for such rare yet significant events in the management of APS. Additionally, this case raises important questions regarding the appropriateness of DOACs in this patient population, especially given the latest recommendations against their use. Increased awareness and further investigation into the implications of anticoagulation strategies in APS patients are warranted to improve patient outcomes and safety.

How to treat patients with thrombotic antiphospholipid syndrome in 2024?

Antiphospholipid syndrome encompasses a range of clinical conditions, particularly thrombotic or obstetrical manifestations, associated with the presence of antiphospholipid antibodies. Managing thrombotic antiphospholipid syndrome in daily clinical practice can be challenging and requires thorough risk stratification and tailored treatment strategies. Primary prophylaxis focuses on improving traditional thrombosis risk factors and, in certain situations, may include low-dose aspirin and / or prophylactic anticoagulants (e.g., low-molecular-weight heparin). The treatment of thrombotic antiphospholipid syndrome primarily involves long-term anticoagulation with vitamin K antagonists. In some cases, a combination of vitamin K antagonists and low-dose aspirin, vitamin K antagonists with international normalized ratio target >3 or a switch to therapeutic doses of low-molecular-weight heparin might be employed. The use of hydroxychloroquine is essential for patients with secondary systemic lupus erythematosus and may be considered for patients with recurrent thrombosis. In other selected situations, the use of immunomodulatory agents can be considered.

Evaluation of Apelin-13 and Apelin-36 Concentration in Women with Primary Unexplained Recurrent Pregnancy Loss

Abstract Background According to the American College of Obstetricians and Gynecologists, recurrent pregnancy loss is a disease, defined as two or more failed pregnancies. Recurrent pregnancy loss is one of the most frustrating and difficult areas of reproductive medicine because up to 50.0% of cases lack clearly defined etiology. Common conditions that have been associated with recurrent pregnancy losses include uterine abnormalities, parental chromosomal aberrations, various endocrine disturbances and the presence of antiphospholipid antibodies. Aim of the Work to evaluate the potential role of apelin-13 and apelin-36 in patients with primary unexplained recurrent early pregnancy loss. Patients and Methods after ethical committee approval and informed consent from the patients, this case control study was conducted at tertiary care hospital at Ain Shams University hospitals from March 2022 to December 2022 and performed on total of 90 women who attended the outpatient antenatal clinics in their first trimester of pregnancy. Results The current study revealed that there were no significant differences between recurrent pregnancy loss group and control group regarding demographic characteristics; age, body mass index and gestational age (p values = 0.582, 0.278, 0.548) respectively. Our study results revealed that Apelin-13 and Apelin-36 were statistically significantly higher in recurrent pregnancy loss group than in control group (p value<0.001). Our results enhance the prognostic significance of high Apelin sub-fragment concentrations in the pathophysiology of unexplained recurrent pregnancy loss and suggests that apelin-13 and/or apelin-36 may be inhibited or decreased by potential drugs for the treatment of unexplained recurrent pregnancy loss. Conclusion the current study investigated serum apelin-13 and apelin-36 concentrations in women with unexplained recurrent pregnancy loss and demonstrated markedly higher serum concentrations compared to healthy pregnant women. We are of the opinion that our findings are not only significant to reveal the role of the Apelin sub-fragments in the pathophysiology of unexplained pregnancy loss; but also, they will provide information as a reference for the future studies, which will investigate the treatment strategies for the treatment unexplained pregnancy loss, and its associated complications in pregnancy.

Right Ventricular Outflow Tract Mass as the Initial Presentation of Primary Antiphospholipid Syndrome

Abstract Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the presence of antiphospholipid antibodies and clinical manifestations, including thrombosis and pregnancy-related complications. While cardiac manifestations such as intracardiac thrombi, valvular disease, and intracardiac masses have been reported, the presentation of an intracardiac mass as the initial manifestation of primary APS (PAPS) is relatively uncommon. This case report describes a 40-year-old female patient who presented with an echogenic mass in the right ventricular outflow tract, subsequently diagnosed as a calcified amorphous tumor. Further investigation revealed elevated levels of anticardiolipin antibodies and beta-2 glycoprotein leading to the diagnosis of PAPS. The patient later developed digital ischemia, strengthening the suspicion of PAPS. This case highlights the importance of considering APS as a potential underlying cause for intracardiac masses, even in the absence of other typical manifestations. Early recognition and appropriate management, including anticoagulation and immunosuppressive therapy, are crucial to prevent further thrombotic complications and improve patient outcomes.

A Genome-Wide Association Study Suggests New Susceptibility Loci for Primary Antiphospholipid Syndrome.

Primary antiphospholipid syndrome (PAPS) is a rare autoimmune disease characterized by the presence of antiphospholipid antibodies and the occurrence of thrombotic events and pregnancy complications. Our study aimed to identify novel genetic susceptibility loci associated with PAPS. We performed a genome-wide association study comprising 5,485 individuals (482 affected individuals) of European ancestry. Significant and suggestive independent variants from a meta-analysis of approximately 7 million variants were evaluated for functional and biological process enrichment. The genetic risk variability for PAPS in different populations was also assessed. Hierarchical clustering, Mahalanobis distance, and Dirichlet Process Mixtures with uncertainty clustering methods were used to assess genetic similarities between PAPS and other immune-mediated diseases. We revealed genetic associations with PAPS in a regulatory locus within the HLA class II region near HLA-DRA and in STAT1-STAT4 with a genome-wide level of significance; 34 additional suggestive genetic susceptibility loci for PAPS were also identified. The disease risk allele near HLA-DRA is associated with overexpression of HLA-DRB6, HLA-DRB9, HLA-DQA2, and HLA-DQB2 in immune cells, vascular tissue, and nervous tissue. This association is independent of the association between PAPS and HLA-DRB1*1302. Functional analyses highlighted immune-related pathways in PAPS-associated loci. The comparison with other immune-mediated diseases revealed a close genetic relatedness to neuromyelitis optica, systemic sclerosis, and Sjögren syndrome, suggesting co-localized causal variations close to STAT1-STAT4, TNPO3, and BLK. This study represents a comprehensive large-scale genetic analysis for PAPS and provides new insights into the genetic basis and pathophysiology of this rare disease.

Antiphospholipid Syndrome and Catastrophic Antiphospholipid Syndrome: A Comprehensive Review of Pathogenesis, Clinical Features, and Management Strategies.

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the presence of antiphospholipid antibodies (aPLs) that predispose individuals to thrombotic events and pregnancy-related complications. APS can occur as a primary condition or in association with other autoimmune diseases, most commonly systemic lupus erythematosus (SLE). Catastrophic APS (CAPS) is a rare, severe variant of APS, marked by rapid-onset, widespread thrombosis leading to multi-organ failure, often triggered by infections, surgical procedures, or cessation of anticoagulation therapy. Both APS and CAPS present significant clinical challenges due to their potential for severe morbidity and mortality. This comprehensive review aims to provide a detailed overview of the pathogenesis, clinical features, diagnostic criteria, and management strategies for APS and CAPS. The review highlights the immunological mechanisms underlying APS, including the role of aPLs, complement system activation, and endothelial cell dysfunction in developing thrombosis. It also outlines the clinical manifestations of APS, such as venous and arterial thrombosis, pregnancy morbidity, and neurological symptoms, along with the diagnostic criteria based on clinical and laboratory findings. The review delves into its pathogenesis, clinical presentation, and diagnostic challenges in the context of CAPS, emphasizing the need for immediate and intensive therapy to manage this life-threatening condition. Current management strategies for APS, including anticoagulant therapy, immunomodulatory treatments, and specific interventions for pregnancy-related complications, are discussed. The review highlights the importance of a multidisciplinary approach for CAPS, combining anticoagulation, high-dose corticosteroids, plasma exchange, and intravenous immunoglobulin. The review also addresses the prognosis and long-term outcomes for patients with APS and CAPS, underlining the necessity for ongoing monitoring and follow-up to prevent recurrent thrombotic events and manage chronic complications. Finally, future directions in research are explored, focusing on emerging therapies, biomarkers for early diagnosis, and the need for clinical trials to advance the understanding and treatment of these complex syndromes. By enhancing the understanding of APS and CAPS, this review aims to improve diagnosis, treatment, and patient care, ultimately leading to better health outcomes for those affected by these conditions.

O-314 Investigating the embryonic contribution to recurrent pregnancy loss: a retrospective analysis of embryo quality, chromosomal status and morphokinetics in 11,169 embryos

Abstract Study question What is the embryonic contribution, in terms of embryo quality, chromosomal status and morphokinetics, to the reproductive success of recurrent pregnancy loss (RPL) patients? Summary answer RPL patients undergoing PGT-A had significantly fewer embryos available for biopsy, higher aneuploidy rates and showed morphokinetic alterations around the time of blastulation. What is known already RPL is diagnosed following the loss of two or more pregnancies before the 24th week of gestation. RPL has a multifactorial etiology encompassing both maternal and embryonic factors, such as uterine malformations, the presence of anti-phospholipid antibodies, maternal obesity or embryo aneuploidy, among others. While some studies have shown increased rates of chromosomal abnormalities in blastocysts from idiopathic RPL patients, a comprehensive characterization of the embryonic contribution to the disorder remains limited, and the role of embryo morphokinetics is still underexplored. Study design, size, duration This retrospective cohort study analyzed 11,169 embryos from 3,005 PGT-A cycles performed across 9 European fertility clinics between January 2017 and July 2023. Differences in the number of biopsied embryos, aneuploidy rates and embryo quality were compared between RPL and non-RPL patients. Morphokinetic timings were evaluated in a subset of 704 RPL embryos from 2 fertility centers, which was then compared to a separate cohort of embryos (n = 952) derived from double gamete donation cycles (n = 134). Participants/materials, setting, methods Genetic and clinical outcomes were assessed by multivariate analyses adjusting for maternal age, paternal age, the presence of male factor and embryo grade, as appropriate. Kaplan-Meier survival curves were constructed for each morphokinetic parameter (tPNf, t2, t3, t4, t5, t8, tSB and tB) and analyzed by Cox regression using maternal age and male factor as adjusting variables. P-values <0.05 were considered statistically significant. Main results and the role of chance RPL patients undergoing PGT-A represented 15.47% (n = 465/3,005) of the cohort and were significantly younger than the rest of the patients (34,97±6.6 vs 36.38±6.6, p < 0.0001). RPL patients obtained significantly fewer blastocysts available for biopsy (coefficient -0.39, 95%CI: -0.703- -0.076, p = 0.01) and had significantly higher aneuploidy rates (OR = 1.19, 95%CI: 1.06-1.35, p = 0.004). However, embryo quality (excellent vs. rest, p > 0.05) was comparable between the two groups (n = 11,169 embryos). As anticipated, biochemical and clinical pregnancy rates were comparable between RPL patients and non-RPL patients (OR = 0.94, 95%CI:0.72-1.24, p = 0.68; OR = 0.95, 95%CI: 0.72-1.24, p = 0.71, respectively), however patients with RPL presented with significantly higher miscarriage rates (OR = 1.66, 95% CI:1.03-2.74, p = 0.04). Compared to double donation embryos, RPL embryos exhibited significant delays in all analyzed times (tPNf: 23.7±3.1 vs. 22.7±3.1; t2: 26.5±4.1 vs. 25.6±3.4; t3: 37.1±5.4 vs. 36.0±4.7; t4: 39.2±6.3 vs. 37.9±5.3; t5: 50.2±7.9 vs. 49.3±7.4; tsB: 99.4±10.3 vs. 97.9±9.7; tB: 110.3±11.5 vs. 106.4±11.8; p < 0.05). Notably, delays in blastocyst formation were also observed when adjusting for maternal age and male factor (Hazard Ratio=0.38, 95% CI: 0.18-0.79, p = 0.009). Significantly, morphokinetic alterations persisted even when exclusively comparing euploid RPL embryos to the double donation group (n = 41, t8: Hazard Ratio=1.78, 95% CI: 1.22-2.57, p = 0.002; tsB: Hazard Ratio=1.70, 95% CI: 1.17-2.48, p = 0.006). Limitations, reasons for caution The main limitation of the study is its retrospective design, where known RPL risk factors leading to the selection of a PGT-A cycle were not considered, potentially introducing biases. Wider implications of the findings These findings highlight the intricate interplay between embryo quality, aneuploidy, and morphokinetics in RPL, guiding personalized patient management. Higher embryo aneuploidy and morphokinetic alterations observed even in euploid RPL embryos suggest an embryonic role, underscoring the condition’s multifactorial nature and urging exploration of molecular mechanisms linking embryonic factors to RPL. Trial registration number not applicable

A retrospective study of the prevalence of isolated prolongation of activated partial thromboplastin time in the preoperative setting

BackgroundIsolated prolongation of activated partial thromboplastin time (APTT) has various causes including inheritable bleeding disorders, and has medical significance as it can lead to the cancelation of surgery. However, even an emergency surgery can be conducted in a patient presenting with severe APTT prolongation, provided careful evaluation and appropriate measures are taken. Hence, the identification of the underlying etiology of the prolonged APTT is crucial. To date, little evidence exists regarding the prevalence of isolated APTT prolongation in Japanese patients undergoing surgery. Herein, we aimed to clarify the prevalence of isolated prolongation of APTT in the preoperative setting and to identify the reasons underlying isolated, severely prolonged APTT.MethodsPreoperative coagulation data of all elective and emergent patients who presented to the anesthetic department between January 1, 2020, and June 30, 2023, were retrospectively collected. Isolated prolongation of APTT was defined as an APTT ≥ 37 s with an international normalized ratio of prothrombin time < 1.2. The underlying etiology of the patient with isolated, severely prolonged APTT (≥ 46 s) was investigated, and canceled surgical procedures in relation to the isolated APTT prolongation were searched.ResultsOverall, 10,684 measurements from 9413 patients were included, of which 725 (6.8%) were identified as having isolated APTT prolongation. The reasons for the severely prolonged APTT (n = 60) were miscellaneous, with the most frequently detected etiology being antiphospholipid antibody positivity. Preoperative isolated APTT prolongation contributed to the cancellation of surgery in elective five cases.ConclusionsWe clarified the prevalence of preoperative isolated prolongation of APTT. The presence of antiphospholipid antibody was the most frequently detected etiology of the patient with isolated, severely prolonged APTT. The present study provides an important dataset regarding the isolated prolongation of APTT in East Asian patients undergoing surgery.

Імунологічні особливості у вагітних із великими акушерськими синдромами

Aim of the research – to evaluate the adaptive capabilities of the immunological system in pregnant women who had complications from the group of major obstetric syndromes (МОS) for the further development of an algorithm for the prevention of this complication. Materials and methods. The main study group (MG) - 99 pregnant women who had a complication from the group of MOS, which was clinically manifested by placental insufficiency (PI); control group (CG) - 50 practically healthy pregnant women with a healthy reproductive history and an uncomplicated course of this pregnancy. The number (x109/l) and relative (%) of lymphocyte subpopulations CD3+ (T-lymphocytes), CD4+ (helper-inducers), CD8+ (cytotoxic suppressors), CD56+ (natural killers), CD19+ (B-lymphocytes) were determined; the presence of antiphospholipid antibodies (APA); total hemolytic activity of the classical way of complement activation (CH50); levels of IgG, IgM, IgA. Statistical processing of research results was carried out using standard Microsoft Excel 5.0 and Statistical 6.0 programs. Results. In the I and II trimesters of pregnancy in MG and CG, a tendency to decrease was observed, СM4+ (51.8±2.5% and 48.9±1.6; 58.4±2.3% and 55.6±2.6%), and СD8+ (24.6±2.4% and 21.2±1.2%; 31.3±2.5% and 30.9±2.7%), which led to an increase in the immunoregulatory index (СD4+/СD8+) in MG (р&lt;0.05). The level of СD19+, during gestation there was an increase in their relative number in MG (13.6±0.98%; 16.4±2.03% and 21.1±2.01%) (p&lt;0.05); a predominance of the percentage of CD56+ lymphocytes, which have the properties of natural killers, was observed in pregnant MG (12.3±1.7%; 15.1±1.7%; 13.9±1.73% vs. 8.6±1.4%; 8.1±1.18%; 7.2±0.98%; p&lt;0.05). Hemolytic activity of the classical pathway of complement activation (CH50) in MG pregnant women was 1.5 times higher compared to CG pregnant women (p&lt;0.05). APA, represented by IgG to cardiolipin, were detected in 47 (47.5%) pregnant women MG and 4 (8.0%) women pregnant CG (p&lt;0.05). Conclusions. Іn pregnant women with МОS, there is an immune maladaptation that progressively increases in the dynamics of gestation and is manifested by excessive activation of natural killers (CD56+), an increase in the immunoregulatory coefficient (CD4+/CD8+), an increase in the percentage of B-lymphocytes (CD19+), dysimmunoglobulinemia, which is accompanied by high indicators of the general hemolytic activity of the classical pathway of complement activation (CH50), the presence of APA. The research was conducted according to principles of Declaration of Helsinki. Protocol of research was proved by local ethical committee, mentioned in institution’s work. A informed sonsennt was collected in order to carry out the research. The author is stating no conflict of interests is declared.

Antiphospholipid Antibodies Are Major Risk Factors for Non-Thrombotic Cardiac Complications in Systemic Lupus Erythematosus.

In systemic lupus erythematosus (SLE), cardiovascular complications are among the leading causes of death. Cardiovascular risk in SLE is even higher in the presence of antiphospholipid antibodies or secondary antiphospholipid syndrome (APS). The aim of this retrospective, single-center study was to investigate the occurrence of antiphospholipid antibodies and non-thrombotic cardiac manifestations in 369 SLE patients. We also assessed the clinical and laboratory characteristics of the patients to reveal the risk factors for cardiac manifestations. Patients were divided into two groups based on the presence of antiphospholipid antibodies (APA); 258 (69.9%) patients were APA positive, and 111 (30.1%) patients were APA negative. Mitral and tricuspid insufficiency, aortic stenosis and pulmonary arterial hypertension were more common in APA-positive patients. Anticardiolipin IgG showed the strongest correlation with any non-thrombotic cardiac manifestations. Based on our results, the adjusted global antiphospholipid syndrome score (aGAPSS) above 8.5 is predictive of valvulopathies and ischemic heart disease, while aGAPSS above 9.5 is predictive of cardiomyopathies. The presence of antiphospholipid antibodies may affect the development of cardiac manifestations in SLE. Periodic cardiological and echocardiographic screening of patients without cardiac complaints, as well as regular monitoring of antiphospholipid antibodies, have great importance during the treatment of SLE patients.

Autoimmune inner ear disease associated with antiphospholipid antibodies.

The percentage of autoimmune diseases in Western countries is approximately 8% of the total population. Despite numerous studies indicating an increase in prevalence and incidence over the past two decades, autoimmune vestibular disorders seem to be underdiagnosed, primarily due to the lack of a definitive test capable of identifying the specific antigen of the inner ear. Autoimmune inner ear disease (AIED) is defined as a rapidly progressive and often fluctuating bilateral neurosensorial hearing loss that develops over a period of weeks or months. AIED can affect only the inner ear or be part of systemic diseases such as granulomatosis with polyangiitis, Cogan's syndrome, systemic lupus erythematosus, polyarteritis nodosa, or relapsing polychondritis, among others. Our main objective was to conduct a study on the presence of antiphospholipid antibodies (aPL) in patients with AIED seen in a specialised clinic. We designed an observational retrospective study in which we selected patients from a total group of 55 with AIED referred to the Autoimmune Diseases Clinic, those with confirmed positivity for antiphospholipid antibodies, and described their clinical, analytical, and epidemiological characteristics. We found a prevalence of 29% positivity for antiphospholipid antibodies, with lupus anticoagulant (LA) being the most frequently detected, followed by anticardiolipin (aCL) and anti-beta2 glycoprotein (anti-B2GP). Double positivity was observed in 25% of patients. The main clinical manifestations were bilateral hearing loss, vestibular symptoms, and tinnitus. Only 25% of patients experienced audiometric improvement during the course of the disease. We emphasise the importance of identifying the presence of aPL in AIED, enabling the establishment of appropriate and specific therapeutic management to prevent audiometric deterioration.

Systemic risk factors of retinopathy in patients with systemic lupus erythematosus.

To investigate the risk factors of lupus retinopathy (LR) in patients with systemic lupus erythematosus (SLE). This is a retrospective, cross-sectional study. LR patients admitted at Peking Union Medical College Hospital from June 2013 to April 2023 were reviewed. Age- and gender-matched SLE patients without retinopathy were selected as controls. Medical records including clinical manifestations, laboratory data and ophthalmic examination were collected. Univariate and multivariate logistic regression analyses were conducted. One hundred and twelve LR patients (198 eyes) were included, with 12 cases (14 eyes) presenting with retinal macrovascular obstruction, and 100 cases (184 eyes) only exhibiting microvasculopathy. Multivariate analysis indicated the presence of haemolytic anaemia, decreased haemoglobin (HGB) and higher relative percentage of neutrophils were independent risk factors for LR (p < 0.05). The first two were also risk factors for retinal microvasculopathy, whereas secondary antiphospholipid syndrome (APS) was for macrovascular obstruction. In male group, LR had significant associations with decreased HGB, no matter which types of retinopathy (p < 0.05). In female group, LR was significantly associated with haemolytic anaemia, presence of antiphospholipid antibodies, decreased white blood cells and relative high percentage of neutrophils. Specifically, haemolytic anaemia (p = 0.002) was significantly associated with retinal microvasculopathy, and APS (p = 0.003) was significantly associated with macrovasculature obstruction. LR was related to haemolytic anaemia, decreased HGB levels and higher percentage of neutrophils. Retinal microvasculopathy accounted for most cases and macrovasculature obstructions were rare. Male and female patients have distinct risk factors. Early ophthalmic screening is recommended especially for those with risk factors of LR.

A mysterious hematochezia in an adolescent boy: atypical presentation of childhood systemic lupus erythematosus

Thrombosis is a well-known entity in presence of antiphospholipid antibody (APLA) associated with systemic lupus erythematosus (SLE) as a hematological complication. Bleeding manifestations instead of thrombosis is hardly found in literature in presence of APLA seromarkers in SLE. Since these can range from minor bleeding like epistaxis to major life-threatening intracranial bleeding, early diagnosis and prompt treatment is essential to manage such condition. We report a 12 years old boy with no significant past history presented with hematochezia and epistaxis along with pallor requiring blood transfusion. Hematological investigations were normal except for elevated PT, aPTT and INR. Common causes of coagulopathy were ruled out. Upon suspecting systemic diseases, the investigations were carried out which revealed ANA 4+ along with high titre of dsDNA, low C3 and C4 complement and positive anti-β2 glycoprotein, anticardiolipin antibody and lupus anticoagulant. Diagnosis of SLE was made according to ACR-EULAR criteria with no renal involvement. Immunological basis was considered for coagulopathy in this child. He was started on oral prednisolone, hydroxychloroquine and methotrexate. He is now under close monitoring of the coagulation profile for titration of steroid dose. We want to create awareness about the uncommon hematological manifestation of SLE presenting as bleeding diathesis instead of thrombosis through this case report and that can be life threatening too if not treated promptly. A high index of suspicion and careful follow-up may help in preventing adverse outcome of the disease.

Clinical Case of Suspected Caecal Appendicitis Concealing Right Renal Artery Thrombosis

We describe an Equadorean patient, 48 years old, who registered to the Emergency Room with pain, fever, leukocytosis, and c-reactive protein in the right side and right iliac fossa and was admitted for suspected acute appendicopathy. Subjected to abdominal CT scan, the presence of a right renal infarct area confirmed by angiographic study is detected. Admitted to Vascular Surgery, he was transferred to Medicine and detected to be double positive for phospholipid antibodies, concomitantly elevated triglyceride values were found. The patient, treated during hospitalization with heparin, was discharged with Warfarin-based oral anticoagulant therapy being contraindicated with direct coagulation inhibitors in the presence of antiphospholipid antibodies.

Single-cell transcriptome analyses reveal disturbed decidual homoeostasis in obstetric antiphospholipid syndrome

ObjectivesObstetric antiphospholipid syndrome (OAPS) is an autoimmune disease characterised by the presence of antiphospholipid antibodies in circulation and pathological pregnancy. However, the pathogenesis of OAPS remains unknown. We aimed to...

Platelets and Thrombotic Antiphospholipid Syndrome

Antiphospholipid antibody syndrome (APS) is an autoimmune disorder characterised by thrombosis and the presence of antiphospholipid antibodies (aPL): lupus anticoagulant and/or IgG/IgM anti-β2-glycoprotein I and anticardiolipin antibodies. APS carries significant morbidity for a relatively young patient population from recurrent thrombosis in any vascular bed (arterial, venous, or microvascular), often despite current standard of care, which is anticoagulation with vitamin K antagonists (VKA). Platelets have established roles in thrombosis at any site, and platelet hyperreactivity is clearly demonstrated in the pathophysiology of APS. Together with excess thrombin generation, platelet activation and aggregation are the common end result of all the pathophysiological pathways leading to thrombosis in APS. However, antiplatelet therapies play little role in APS, reserved as a possible option of low dose aspirin in addition to VKA in arterial or refractory thrombosis. This review outlines the current evidence and mechanisms for excessive platelet activation in APS, how it plays a central role in APS-related thrombosis, what evidence for antiplatelets is available in clinical outcomes studies, and potential future avenues to define how to target platelet hyperreactivity better with minimal impact on haemostasis.

P-01 Case report of adrenal insufficiency due to bilateral adrenal hemorage

Abstract Introduction Arterial or venous thrombosis may be seen in the presence of antiphospholipid antibody (AFA). Rarely, it can cause adrenal hemorrhage. In this case, a 39-year-old case of adrenal insufficiency due to adrenal hemorrhage accompanied by AFA is presented. Clinical Case A 39-year-old male patient with no known disease was admitted to the emergency service with complaints of abdominal pain, general condition disorder, weakness, nausea and vomiting. Hypotension, Na:121meq/L (136-145 meq/L) and K:6.2 meq/L (3.5-5 meq/L), creatinine: 3.56 mg/dl (0.7-1.20), urea: 50.5 mg /dl (6-20), cortisol value measured by suspecting acute adrenal insufficiency was 1.42 µg/dL (5-25 µg/dL), ACTH value was 561 pg/mL (10-46 pg/mL). Abdominal CT for abdominal pain showed a collection area of 58 mm ap diameter on the right and 66 mm ap diameter on the left in both surrenal lobes, and numerous millimeter-sized hypointense areas in the subcapsular area of both kidneys and were evaluated as significant in terms of microinfarcts. Then, areas that were hyperdense in the precontrast series and did not show significant enhancement in the postcontrast series were evaluated for bleeding in adrenal CT (Figure 1).In the tests performed, aPTT 44.5 sec (24-40), C3 1.272 g/dL (0.9-1.8 g/dL), ANA:1/160 homogeneous positive granular, anti-dsDNA:28 IU/mL, lupus anticoagulation positive, in complete urinalysis proteinuria and hyaline cast were detected. The patient was diagnosed with AFAS and systemic lupus erythematosus (SLE), and hydroxychloroquine was started. Warfarin and acetylsalicylic acid were started due to renal vein thrombosis. Stress-dose steroid therapy (80 mg/day methylprednisolone) was started in the patient who was suspected of having adrenal insufficiency due to adrenal hemorrhage. The hemorrhage in our patient was attributed to the hemorrhage caused by the AFA-related infarction. The patient who completed the treatment was discharged with 200 mg hydroxychloroquine, 30 mg hydrocortisone, 0.5 mg fludrocortisone, 5 mg warfarin, 100 mg acetylsalicylic acid, 10 mg amlodipine. Conclusion Although the pathogenetic mechanism of adrenal insufficiency seen in AFA syndrome is not fully known; increased coagulation status in these patients is held responsible. Adrenal insufficiency is thought to be due to hemorrhage after venous thrombosis at the adrenal vein or microvascular level. AFA-related adrenal insufficiency is usually permanent and rarely resolves. Therefore, long-term follow-up of these patients is required. The presence of AFA should be investigated in patients presenting with bilateral adrenal hemorrhage.Figure 1.Sürrenal bt

Catastrophic Antiphospholipid Syndrome.

Unlike classic APS, CAPS causes multiple microthrombosis due to an increased inflammatory response, known as a "thrombotic storm". CAPS typically develops after infection, trauma, or surgery and begins with the following symptoms: fever, thrombocytopenia, muscle weakness, visual and cognitive disturbances, abdominal pain, renal failure, and disseminated intravascular coagulation. Although the presence of antiphospholipid antibodies in the blood is one of the diagnostic criteria, the level of these antibodies can fluctuate significantly, which complicates the diagnostic process and can lead to erroneous interpretation of rapidly developing symptoms. Triple therapy is often used to treat CAPS, which includes the use of anticoagulants, plasmapheresis, and high doses of glucocorticosteroids and, in some cases, additional intravenous immunoglobulins. The use of LMWH is recommended as the drug of choice due to its anti-inflammatory and anticoagulant properties. CAPS is a multifactorial disease that requires not only an interdisciplinary approach but also highly qualified medical care, adequate and timely diagnosis, and appropriate prevention in the context of relapse or occurrence of the disease. Improved new clinical protocols and education of medical personnel regarding CAPS can significantly improve the therapeutic approach and reduce mortality rates.

Adjusted Global Antiphospholipid Syndrome Score (aGAPSS) and PLT, APTT: Predictive Value of Thrombotic Risk Assessment in SLE Patients.

Risk assessment of vascular thrombosis in SLE patients with the presence of antiphospholipid antibodies (aPL) remains a challenge. The adjusted global antiphospholipid syndrome score (aGAPSS) has been validated and used to predict aPL-related thrombosis in SLE patients in some countries. Relevant data of aGAPSS in thrombotic evaluation in SLE population from China has not been reported. We aim to validate aGAPSS in thrombosis assessment in Chinese patients with SLE and to explore the correlations of aGAPSS with routine laboratory parameters and their clinical significance as well. A total of 166 consecutive SLE patients were retrospectively analyzed. Multivariate logistic regression analysis was performed to examine the impact of multiple cardiovascular risk factors and laboratory parameters in recurrent thrombosis risk in SLE. ROC was conducted to explore the discriminative ability of aGAPSS and platelet (PLT), activated partial thromboplastin time (APTT), alone or in combination. Significantly higher value of aGAPSS was seen in SLE patients with vascular thrombosis. ROC curve indicated that aGAPSS of 3.5 or more had the best diagnostic accuracy for the prediction of aPL-related thrombosis in SLE patients. PLT with cutoff of 187.5 x 109/L and APTT with 37.5 seconds were predictors of aPL-related thrombosis as well. The combination of aGAPSS with PLT and APTT improved AUC compared to aGAPSS alone. The aGAPSS could predict the risk of aPL-related vascular thrombosis in SLE patients from China. The combination of aGAPSS with PLT and APTT was first time proved to have better predictive performance in thrombosis risk assessment in SLE.

Association of anti-phospholipid antibodies and hepatitis B virus infection in Najaf Governorate

Abstract Background: Antiphospholipid syndrome (APS) is an autoimmune disease with multifactorial risk factors including genetic and environmental factors such as viral infection, and it can be diagnosed by the presence of antiphospholipid antibodies (APLA) in the serum. Hepatitis B virus (HBV) is one of the most prevalent viral infections in the world and has been connected to a number of autoimmune illnesses. Some studies suggested that HBV might be the cause of APLA production and APS development. No previous study was done in Iraq regarding the relationship between these two. Objective: To estimate the prevalence of APLA in HBV-infected individuals in Najaf. Materials and Methods: A cross-sectional study was done in Najaf from September 2022 to March 2023, and it included 113 patients (70 were males, age range was 15–85 years) with HBV infection and without any history of APS. They were tested for Hepatitis B core (HBc) total and immunoglobulin M (IgM) Abs and then tested for APLA by enzyme-linked immunosorbent assay (ELISA) technique. The Statistical Program for Social Sciences (SPSS) version 26.0 was used for statistical analysis. Results: Only four patients were positive for HBc total and IgM the remaining 109 were positive for IgG only, 38 (33.63%) were positive for APLA, and the age group (41–50 years) with the highest rate of APLA positivity. Non-significant statistical difference was seen between APLA and gender or age. Acute HBV had higher titers of APLA. Conclusion: HBV may lead to the production of APLA in some patients without any other risk factors, especially in middle-aged patients.