Incidence Of Antiphospholipid Syndrome Research Articles

Epidemiology of Antiphospholipid Syndrome in the General Population.

Purpose of ReviewThe epidemiology of antiphospholipid syndrome (APS) is poorly understood. Here, we review the current understanding of the epidemiology of antiphospholipid syndrome in the general population and the frequency of antiphospholipid antibodies in the general population in patients with obstetric morbidity, arterial events, and venous thromboembolism.Recent FindingsThere have been few population-based studies that estimated the prevalence and incidence of APS. The estimated incidence and prevalence among most these studies ranged between 1 and 2 cases per 100,000 and 40 and 50 cases per 100,000 respectively. The prevalence of antiphospholipid antibodies in patients with obstetric morbidity was 6–9%, while in arterial events and venous thromboembolism is 9–10%. However, this data remains limited. Mortality of patients with APS is 50–80% higher than the general population.SummaryThe epidemiology of APS has been difficult to elucidate. Population-based studies patients with diverse age, racial, and ethnic backgrounds are needed.

An Update on Antiphospholipid Syndrome.

To review the recent available evidence on epidemiology, pathogenesis, clinical phenotypes, and management of antiphospholipid syndrome (APS) and summarize potential future research perspectives. Accumulating evidence has further expanded our understanding of the disease, including new data about the incidence and prevalence of APS, novel pathways supporting the role of thrombo-inflammation in APS including platelet, monocyte and endothelial cell activation, pro-inflammatory cytokine and chemokine production, complement activation, neutrophil extracellular trap release, and type I interferon gene expression that could yield to new potential treatment targets, better identification of criteria and non-criteria clinical phenotypes, antiphospholipid antibody profiles and their associations with clinical outcomes, prognostic tools, and treatment strategies based on recent evidence-based recommendations for patients with thrombotic and obstetric APS, with or without systemic lupus erythematosus. Ongoing research efforts and international collaborations enhance our knowledge of this rare and often devastating syndrome and help improve patient care and health outcomes.

Cardiac Surgery in a Patient With Antiphospholipid Syndrome and Heparin-Induced Thrombocytopenia

ANTIPHOSPHOLIPID SYNDROME (APS) and heparin-induced thrombocytopenia (HIT) are separate disease processes that are both characterized by a prothrombotic state and an increased risk of perioperative complications. APS is a rare, noninflammatory, autoimmune disease, resulting in arterial and/or venous thrombosis, thrombocytopenia, fetal loss, and the presence of antiphospholipid (APL) antibodies. 1 Mishra PK Khazi FM You P et al. Severe antiphospholipid syndrome and cardiac surgery: Perioperative management. Asian Cardiovasc Thorac Ann. 2016; 24: 473-476 Crossref PubMed Scopus (4) Google Scholar The incidence of APS is approximately 2% in the general population. 2 Koniari I Siminelakis SN Baikoussis NG et al. Antiphospholipid syndrome: Its implication in cardiovascular diseases: A review. J Cardiothorac Surg. 2010; 5: 101 Crossref PubMed Scopus (72) Google Scholar Irregular thickening of heart valves from immune-complex deposition leads to valvular disease in 35% to 82% of patients with APS, and 4% to 6% of these patients require surgical intervention. 3 Berkun Y Elami A Meir K et al. Increased morbidity and mortality in patients with antiphospholipid syndrome undergoing valve replacement surgery. J Thorac Cardiovasc Surg. 2004; 127: 414-420 Abstract Full Text Full Text PDF PubMed Scopus (55) Google Scholar The mitral valve is the most commonly affected, followed by the aortic valve. 1 Mishra PK Khazi FM You P et al. Severe antiphospholipid syndrome and cardiac surgery: Perioperative management. Asian Cardiovasc Thorac Ann. 2016; 24: 473-476 Crossref PubMed Scopus (4) Google Scholar Due to the prothrombotic state, valve surgery mortality rates are as high as 10% to 20%, 4 Nakajima T Enomoto Y Ishigaki M et al. Perioperative anticoagulation management during aortic valve replacement complicated by antiphospholipid syndrome. J Card Surg. 2017; 32: 633-635 Crossref PubMed Scopus (4) Google Scholar and surgical intervention usually is deferred to medical management if possible. 5 Erkan D Yazici Y Sobel R et al. Primary antiphospholipid syndrome: Functional outcome after 10 years. J Rheumatol. 2000; 27: 2817-2821 PubMed Google Scholar

28-Year-Old Man With Recurrent Vertigo, Syncope, and Progressive Memory Impairment

28-Year-Old Man With Recurrent Vertigo, Syncope, and Progressive Memory Impairment

Antifospholipid Syndrome with Autoimun Hemolitic Anemia

Abstract: Antiphospholipid syndrome is a part of systemic autoimmune disease (nonspecific organ), characterized by vascular thrombosis (arterial or venous) with a high antibody titter against a plasma protein that binds to phospholipid anions (antiphospholipid-aPL antibodies). Autoimmune haemolytic anaemia (AIHA) is rare in patients with antiphospholipid antibody syndrome. A sixteen years old female patient has been reported with the main complaint of swelling in the right leg. The patient also complained of increased fatigue. On physical examination found anaemic conjunctiva, swelling in the right limb with a diameter of the right leg greater than the left leg. Homan’s sign positive on the right leg. This patient belongs to the high probability criteria of DVT. Doppler Ultrasonography (USG) examination suggests thrombus in the iliac vein, femoral vein, and right leg popliteal vein, and thrombus in the left leg popliteal vein. In the antibody anticardiolipin (ACA) IgG examination an increase was found. The comb test was positive, and haemolytic anaemia antibody screening was the warm type. Heparinisation and immunosuppressant administration were performed in patients. The incidence of antiphospholipid syndrome with AIHA cannot be ascertained whether there is a direct relationship. Keywords: antiphospholipid syndrome, DVT, AIHA

Epidemiology of Antiphospholipid Syndrome in Korea: a Nationwide Population-based Study.

BackgroundAntiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and venous thrombosis or pregnancy morbidity in patients with persistent antiphospholipid antibodies. However, nationwide population-based epidemiology studies regarding APS are still unavailable.MethodsWe analyzed claims data extracted from the Korean Health Insurance and Review Agency (HIRA) covering more than 52 million Koreans, between January 1, 2008, and December 31, 2017. Patients diagnosed with APS, as determined by the Korean Classification of Disease, 7th edition (D68.6), and a rare intractable disease program (V253), were identified in HIRA.ResultsA total of 3,088 newly diagnosed incident cases of 1,215 men and 1,873 women were identified during 2009–2016. The mean age was 44.6 ± 16.6 (men, 47.4 ± 16.3; women, 42.8 ± 16.6) years. The incidence was 0.75 per 105 person-year (95% confidence interval, 0.73–0.78). The prevalence in 2016 was 6.19 per 105 people. For incident cases, women showed incidence peak at ages of 30–39 years and 70–79 years, whereas for men, it was highest at ages of 70–79 years only. Of all patients, 1,766 (57%, 810 men and 956 women) had primary APS, 1,322 (43%, 405 men and 917 women) had secondary APS, and 845 (27%, 216 men and 629 women) were associated with systemic lupus erythematosus (SLE).ConclusionThe incidence of APS differs according to age groups and gender. The incidence of primary APS was higher than that of secondary APS in both gender. Furthermore, as already reported, secondary APS is highly associated with SLE; however, we observed that rheumatoid arthritis is also highly related.

The Epidemiology of Antiphospholipid Syndrome: A Population-Based Study.

To estimate the annual incidence and prevalence of and frequency of mortality associated with antiphospholipid syndrome (APS). An inception cohort of patients with incident APS in 2000-2015 from a geographically well-defined population was identified based on comprehensive individual medical records review. All cases met the 2006 Sydney criteria for APS (primary definition) or had a diagnosis of APS confirmed by physician consensus (secondary definition). Levels of lupus anticoagulant, IgM and IgG anticardiolipin antibodies, and anti-β2-glycoprotein I antibodies were tested in a centralized laboratory. Incidence rates were age- and sex-adjusted to the 2010 US white population. Prevalence estimates were obtained from the incidence rates, assuming that there was no increased mortality associated with APS and that migration in or out of the area was independent of disease status. Among this cohort in 2000-2015, 33 cases of incident APS, as defined by the Sydney criteria, were identified (mean age of patients 54.2 years; 55% female, 97% white). The annual incidence of APS in adults ages ≥18 years was 2.1 (95% confidence interval [95% CI] 1.4-2.8) per 100,000 population. Incidence rates were similar in both sexes. The estimated prevalence of APS was 50 (95% CI 42-58) per 100,000 population, and was similar in both sexes. Six patients (18%) had a concurrent diagnosis of systemic lupus erythematosus. The most frequent clinical manifestation was deep vein thrombosis. The overall frequency of mortality among patients with APS was not significantly different from that in the general population (standardized mortality ratio 1.61, 95% CI 0.74-3.05). APS occurred in ~2 persons per 100,000 population per year. The estimated prevalence was 50 per 100,000 population. Overall mortality was not notably different from that observed in the general population.

Effect of Thrombophilic Factors on Renal Graft Function: A Single-Center Experience

BackgroundOptimization of immunosuppressive therapy reduced the incidence of acute rejection, and therefore vascular complications, including graft thrombosis, which have emerged as the main cause of graft loss in the early post-transplant period. A thrombophilic condition may lead to renal graft loss. The aim of the study was to assess renal graft function in thrombophilic renal recipients receiving anticoagulation treatment. MethodsThis is a retrospective study including 29 renal recipients (ktx group) with a history of thrombosis and confirmed thrombophilic factor. Graft function was evaluated by median serum creatinine concentration at the third month after ktx (SCr1) and at the end of the observation (SCr2) with respect to hypercoagulability (factor V Leiden [FVL], mutation G20210A, antiphospholipid antibodies, deficiency of protein S [PS] or C [PC], factor VIII >200%). ResultsRecipients underwent retransplantation because of graft thrombosis (P < .001). They more often underwent urgent transplantation (P = .008), received induction therapy (P = .021), underwent an indication other than protocol biopsy (P = .001), or experienced acute rejection (P = .042). Differences in graft function (SCr2) were found at the end of observation (ktx group vs controls 1.9 mg/dL vs 1.3 mg/dL, respectively, P = .014). Multivariate analysis revealed inferior thrombophilic graft function in the model with SCr1 <2 mg/dL (odds ratio 0.07, 95% confidence interval 0.01–0.57, P = .014) and in the model with SCr2 <2 mg/dL (odds ratio 0.15; 95% confidence interval 0.04–0.54, P = .004). The incidence of antiphospholipid syndrome was 31%; FVIII, 31%; FVL, 24.1%; and PC/PS, 13.8%. After anticoagulation was introduced no thromboembolic events or bleeding complications occurred. ConclusionHypercoagulability is not a contraindication to ktx but may worsen graft function. Post-transplant care in thrombophilic recipients is demanding (retransplantation, immunization, protocol biopsy, anticoagulation), but is the only means by which to maintain a graft.

Diagnosis and management of obstetrical antiphospholipid syndrome: where do we stand?

Obstetrical antiphospholipid syndrome (APS) is defined by obstetrical complications and the presence of antiphospholipid antibodies (aPL). Although the incidence of APS is still poorly known, this thrombophilia is now recognized as one of the most common acquired causes of recurrent fetal loss. The diagnosis of APS during pregnancy can be challenging because of its various clinical features. Mothers with APS have an increased risk of thrombosis, thrombopenia, and specific pregnancy‑related complications such as preeclampsia, eclampsia, and hemolysis elevated liver enzyme and low‑platelet syndrome. aPL can also lead to recurrent, early miscarriages, stillbirths, and to intrauterine growth restriction. Clinicians should be aware of all these characteristics and a thorough differential diagnosis should be performed. Testing for aPL also requires skill due to the difficulty of standardization and interpretation of tests. To know when testing should be performed and when to repeat tests are still a matter of debate. While general management and first‑line treatment of APS during pregnancy now have clear guidelines, second‑line treatment is still required in 30% of the cases and new strategies are currently in development. In this review, we describe the clinical and biological aspects of obstetrical APS and its current management options. As APS pregnancies can be a real challenge for clinicians, we underline the necessity of multidisciplinary counselling and close follow‑up.

Does anti-DNA positivity increase the incidence of secondary antiphospholipid syndrome in lupus patients?

Aim of the workTo detect the incidence of secondary antiphospholipid syndrome (APS) among Systemic lupus erythematosus (SLE) patients with positive anti-DNA antibodies. Patients and methodsWe studied 342 SLE patients; Group I: anti-DNA positive SLE patients (n=208) and Group II: anti-DNA negative SLE patients (n=134), with a female to male ratio of 9.39:1 and a mean age of 27.49±7.94years and disease duration of 5.74±3.97years. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed. Disease activity was assessed using systemic lupus erythematosus disease activity index (SLEDAI). Anti-dsDNA tests were carried out by indirect Immunofluorescence (IF) technique. Anti cardiolipin antibodies (IgG and IgM) and Anti-β2 glycoprotein-I antibody of IgG and/or IgM isotype were detected by ELISA. ResultsThe clinical manifestations, disease activity and laboratory investigations of the SLE patients varied according to the anti-DNA antibodies. Thirty-six patients (17.3%) had secondary APS in those with positive anti-DNA antibodies while only16 (11.9%) had secondary APS in those with negative anti-DNA antibodies, with no significant differences between both groups. ConclusionApparent higher incidence of secondary APS was detected in anti-DNA positive SLE patients. The non significant differences between both groups may suggest that anti-DNA positivity cannot be considered as the only predictor of secondary APS and further studies may be needed to detect other factors which may increase the incidence of APS in SLE patients.