432 ACS-STEMI in a young man with triple positive antiphospholipid syndrome: clinical implications about antithrombotic therapy management

Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disorder characterized by venous and arterial thromboembolic (TE) disease, and/or pregnancy morbidity, associated with persistent elevated antiphospholipid antibodies (lupus anticoagulant, LA, anticardiolipin, aCL, and anti-beta2-glycoprotein I, anti-β2GPI). A 51 year-old man, smoker, presented to our ER with chest pain. EKG showed Q waves and STE in the anterior leads, with increased troponin levels. A diagnosis of anterior STEMI was made and he was taken into our catheterization laboratory, where a high burden thrombotic plaque determining LM and ostial LAD subocclusion was treated with PPCI and a DES implantation; in addition, multiple failed PCI attempts were performed on a distal LAD occlusion, and an integrilin bolus was administered. The patient was admitted to our CICU and a DAPT with ASA and Ticagrelor was started. TTE showed LVEF 40%, apical akinesis, septal and anterior hypokinesis, and no significant valve disease. An aPTT abnormal value (100.5 s; reference range 25–38.5 s) was detected, not corrected by aPTT mixing study. Given suspected autoimmune prothrombotic state, normal Hb and PLT values and low bleeding risk, LMWH 100 IU/kg every 12 h was started, in addition to DAPT with switch from Ticagrelor to Clopidogrel. LA and aCL and anti-β2GPI IgG were positive, with negativity of others rheumatologic tests, confirming primary APS diagnosis. Repeat TTE showed EF 50%. The patient was discharged in ‘triple therapy’ (DAPT plus LMWH), and referred to the rheumatology clinic. LA, aCL, and anti-β2GPI tests 12 weeks later were positive, confirming a triple positive APS and satisfying revised Sapporo criteria. The rheumatologist switched therapy to Clopidogrel plus Warfarin. At 3 months follow-up, the patient, former smoker, was asymptomatic and TTE confirmed EF 50%, so he was recommended to continue with his medical therapy. APS is rarely associated with AMI (∼5.5%) and in only 2.8% cases AMI represents the onset of the disease. AMI pathogenesis in APS is considered to be acute thrombosis of coronary arteries, in contrast with atherosclerotic plaque rupture in typical AMI. Key discriminators to identify APS as potential underlying cause of AMI include young age, previous unprovoked thromboses, low platelets count (they are consumed in the thrombotic process), high aPTT value (LA may interfere with assembly of the prothrombinase complex on phospholipids), coronary artery thromboses in the setting of otherwise normal otherwise appearing coronary arteries. APS antibodies have also pro-inflammatory activity on vascular endothelial cells, leading to accelerated atherosclerosis. aGAPSS score (high risk ≥10) is useful for risk stratification of recurrent thrombosis and AMI in young patients with APS. The treatment of AMI in APS is therefore a clinical challenge. Strict management of additional CV risk factors is crucial. VKA (INR >3 with Warfarin, INR2–3 with Warfarin and ASA) should be provided for life, because of the very high risk of recurrent TE. DOACs are less effective and less safe than VKAs for TE prevention in APS. The role of coronary stents, considering higher rates of stent thrombosis after PCI in APS patients triple therapy concomitant risks, requires further studies. Due to lack of large, randomized, prospective studies, there is no clear experts consensus about optimal antithrombotic therapy in secondary prevention after arterial TE. APS patients with STEMI should undergo PCI, usually associated with thrombus aspiration, and in selected cases DES implantation in culprit lesion followed by triple antithrombotic therapy with short-term DAPT and long-term VKA.

Pathophysiology of the Antiphospholipid Antibody Syndrome

The antiphospholipid syndrome is characterized by antibodies directed against phospholipid-binding proteins and phospholipids attached to cell membrane receptors, mitochondria, oxidized lipoproteins, and activated complement components. When antibodies bind to these complex antigens, cells are activated and the coagulation and complement cascades are triggered, culminating in thrombotic events and pregnancy morbidity that further define the syndrome. The phospholipid-binding proteins most often involved are annexins II and V, β2-glycoprotein I, prothrombin, and cardiolipin. A distinguishing feature of the antiphospholipid syndrome is the “lupus anticoagulant”. This is not a single entity but rather a family of antibodies directed against complex antigens consisting of β2-glycoprotein I and/or prothrombin bound to an anionic phospholipid. Although these antibodies prolong in vitro clotting times by competing with clotting factors for phospholipid binding sites, they are not associated with clinical bleeding. Rather, they are thrombogenic because they augment thrombin production in vivo by concentrating prothrombin on phospholipid surfaces. Other antiphospholipid antibodies decrease the clot-inhibitory properties of the endothelium and enhance platelet adherence and aggregation. Some are atherogenic because they increase lipid peroxidation by reducing paraoxonase activity, and others impair fetal nutrition by diminishing placental antithrombotic and fibrinolytic activity. This plethora of destructive autoantibodies is currently managed with immunomodulatory agents, but new approaches to treatment might include vaccines against specific autoantigens, blocking the antibodies generated by exposure to cytoplasmic DNA, and selective targeting of aberrant B-cells to reduce or eliminate autoantibody production.

Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome

Introduction: Asherson's Syndrome, also defined as Catastrophic Antiphospholipid Syndrome (CAPS), represents the most severe manifestation of Antiphospholipid Antibody Syndrome. Rarely, the first CAPS diagnosis is based on macro-thrombotic event as acute limb ischemia.Case Presentation: We present a case of a 65-year-old woman admitted with an acute lower limb arterial ischemia with a complete occlusion of all the three tibial vessels. Three endovascular recanalization procedures were performed contemporary to 48 h intraarterial thrombolysis administration. The patency of tibial arteries was restored with a near-complete absence of digital arteries and microvessel perfusion of the foot. In the following days, an aggressive foot gangrene was established, leading to a major lower-limb amputation. Due to the general clinical status worsening and aggressiveness of ischemic condition, further investigations were performed leading to the diagnosis of an aggressive Asherson's Syndrome that was also complicated by a severe heparin-induced thrombocytopenia. Medical management with a high dose of intravenous steroids and nine sessions of plasma exchange led to a clinical condition stabilization.Conclusion: In our case, the presence of a “sine causa” acute arterial occlusion of a large vessel represented the first manifestation of an aggressive form of Asherson's Syndrome that could represent a fatal disease. Due to the extreme variety of manifestations, early clinical suspicion, diagnosis, and multidisciplinary management are essential to limit the life-threatening consequences of patients.

First Report of Fulminant Budd-Chiari Syndrome Treated by Direct Intrahepatic Portocaval Shunt in Vietnam

Budd-Chiari syndrome (BCS) is a rare disorder caused by hepatic venous outflow obstruction that can lead to acute liver failure proposing liver transplantation or transjugular intrahepatic portosystemic shunt. However, the transjugular intrahepatic portosystemic shunt is not always successful due to the entire hepatic vein thrombosis while transplantation is not unfailingly feasible. In these situations, the direct intrahepatic portosystemic shunt (DIPS) is a viable alternative that may ameliorate portal hypertension in these patients. We described a case of a 21-year-old male with fulminant hepatic failure owning to BCS with a 4-day history of abdominal pain and nausea. Laboratory workup, including viral, autoimmune etiologies JAK2 mutation, Factor V Leiden, antiphospholipid antibody syndrome, was negative. The patient’s clinical status worsened with hepatic encephalopathy stage II despite administering anticoagulation. Thus, the patient underwent urgently DIPS after unaccessible to the creation of a transjugular intrahepatic portosystemic shunt and impossible to transplantation. The patient’s health was improved and discharged. Fulminant Budd-Chiari is a rare disease to be demanding prompt treatment. While transplantation or transjugular intrahepatic portosystemic shunt is failed, the DIPS is considered an alternative candidate associated with clinical improvement.

Cluster-Based Immunotherapy for Patients with Recurrent Abortion Caused by Antiphospholipid Syndrome

In order to diagnose patients with pregnancy and antiphospholipid antibody syndrome, provide early treatment, and effectively reduce the pregnancy outcome of the abnormal pregnancy, the effect of antiphospholipid syndrome (APS) immunotherapy on the incidence of abortion was discussed based on clustering algorithm. We selected 62 cases of APS leading to recurrent miscarriage patients, in the early pregnancy injection of low molecular heparin, intravenous drip proprocyclin, and oral tactics, using B-ultrasound images to observe the pregnancy ending. The results show that the hormone levels in the two groups were different before treatment ( P > 0.05 ); after treatment, the HCG, E2, and P hormone levels in the two groups were significantly improved, and the HCG, E2, and P hormone levels in the observation group were significantly higher than those of the control group ( P < 0.05 ); the abortion rate of patients in the observation group was significantly lower than that of the control group ( P < 0.05 ); the antiphosphorus antibody of the study group was significantly higher than that of the control group. For APS patients, immunotherapy is effective. Antiphospholipid syndrome causes remarkable immunotherapy effect in patients with recurrent miscarriage, effectively improves the clinical symptoms of patients, improves antiprochemical antibody rosion, and improves the patient’s pregnancy outcomes, which is worth promoting.

A Case of Systemic Lupus Erythematosus in a Patient Presenting with Libman-Sacks Endocarditis

Background. Libman-Sacks endocarditis (LSE) is a rare cardiovascular manifestation of systemic lupus erythematosus/antiphospholipid syndrome that is described as a sterile verrucous nonbacterial vegetative lesion. These lesions can cause progressive damage to the heart valves leading to valve surgery. The most common valves to be affected are the aortic and mitral valves. Libman-Sacks endocarditis is associated with malignancies, other systemic diseases like systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS). The majority of LSE patients are usually asymptomatic. Case Summary. We describe a 39-year-old male patient who presented with increasing shortness of breath and pulmonary congestion. He was found to have severe mitral valve regurgitation and mitral stenosis. Transesophageal echocardiogram confirmed the diagnosis of Libman-Sacks endocarditis with thickened mitral valve leaflets with symmetrical mass-like structure causing a restriction in the valve function during both cardiac phases later diagnosed with systemic lupus erythematosus by immunology. The patient was started on diuretics, anticoagulants, angiotensin inhibitors, beta-blockers, and hydroxychloroquine. He underwent successful mechanical mitral valve replacement with a 27 mm St. Jude valve. The mitral valve was found to be grossly thickened with friable tissue and complete amalgamation of the leaflets with subvalvular apparatus. The patient suffered some warfarin adverse effects a year later but did well otherwise. Conclusion. This case demonstrates that Libman-Sacks endocarditis can be the first manifestation of systemic lupus erythematosus. Early and prompt diagnosis of LSE can prevent and lessen the many side effects associated with thromboembolism. Additionally, addressing the underlying disease is key to successful treatment.

A-57 The Neuropsychological Impact of Antiphospholipid Antibody Syndrome: A Case Study

Objective Antiphospholipid Antibody Syndrome (APS), also known as Hughes Syndrome, is an autoimmune condition linked to various adverse medical and neurological outcomes affecting 5 in 100,000. APS results from antibodies (aPL) that attack blood proteins that bind to phospholipids (e.g., 2-glycoprotein I and prothrombin), which can cause blood flow problems, increased risk of blood clots, and recurrent vascular thrombotic events. Research suggests APS may lead to various neurologic/medical issues including memory loss, visual disturbances, and dementia. Method Neuropsychological evaluation of 48-year-old female with triple-positive APS and history of bilateral superior parietal chronic ischemic infarctions, multiple bilateral lacunar infarctions, and bilateral encephalomalacia. Reports progressive cognitive changes (&lt; 1 year). Results Neuropsychological evaluation evidenced low average premorbid functioning and currently, extremely low overall cognitive ability. Memory was variable with significant visual and working memory impairment but preserved delayed recall of contextual information. While verbal abilities were intact, deficits were noted in executive functioning, attention, processing speed, visuomotor, visual–spatial, and fine motor skills. Conclusion This 48-year-old woman’s cognitive profile is consistent with findings in the APS literature and is indicative of an early onset major vascular neurocognitive disorder. Retrospective studies suggest that cognitive deficits often precede somatic symptoms of APS and abnormal neuroimaging findings; she presents atypically, as somatic symptoms and abnormal neuroimaging preceded cognitive decline. This case adds to the limited body of neuropsychological data regarding the effects of APS on cognitive functioning, as the pathogenesis of cognitive impairment in APS is unclear and leads to questions regarding differences in, and trajectories of, cognitive deficits in APS.