Prevalence Of Antiphospholipid Syndrome Research Articles

P152 Very Late Onset Systemic Lupus Erythematosus - Comparison with Younger Patients

Abstract Background/Aims Age has a significant impact on SLE. However, data on very late onset SLE (vlSLE) patients are scarce. We have characterized a large SLE patient cohort, followed for up to 45 years and compared the clinical and serological features at the different ages at diagnosis, focusing on those whose disease began at 60 or later. Methods We conducted an observational cross-sectional study at our SLE clinic. All patients with an SLE diagnosis evaluated from January 1978 to May 2023 were included. Patients were divided into four groups according to age at diagnosis: juvenile SLE (jSLE - <18 years); adult SLE (aSLE - 18-49 years); late SLE (lSLE - 50-59 years); vlSLE (≥ 60 years). Proportions and median values were compared using the X2 and Kruskall-Wallis tests, respectively. The probability of survival was estimated using the Kaplan-Meier method. Results 845 patients were enrolled. jSLE, aSLE, lSLE, and vlSLE groups included 153 (18.1%), 630 (74.6%), 47 (5.56%), and 15 (1.78%) patients, respectively. The mean age at diagnosis in vlSLE patients was 66.7 years. The female-to-male ratio tended to fall in the vlSLE group (4:1; p=0.282). vlSLE patients were mainly caucasian (93.3%; p<0.001) and had the lowest survival time (20.3 years; p<0.001). The most frequent clinical feature at follow-up in all groups was arthritis. Nevertheless, arthritis was least common among vlSLE patients (73.3%; p=0.043). Although malar and/or discoid rash, photosensitivity, and alopecia were less frequent among lSLE and vlSLE patients, the difference was not statistically significant. In contrast, oral ulcers tended to be more frequent in vlSLE patients (33.3%; p=0.537). vlSLE patients more commonly developed Sjögren’s syndrome (SS - 33.3%; p<0.001) and rheumatoid arthritis (RA - 13.3%; p<0.001). The prevalence of anti-phospholipid syndrome did not differ significantly between age groups. Neuropsychiatric involvement, and myositis did not occur among vlSLE patients. At diagnosis, vlSLE patients had the lowest prevalence of positive anti-dsDNA antibodies (26.7%; p=0.010) and low C3 levels (13.3%; p<0.001). Anti-SSA/Ro antibody positivity was also least common among vlSLE patients (26.7%; p=0.045). Although vlSLE patients also tended to have the lowest levels of ANA and anti-SSB/La antibody positivity, the difference was not statistically significant. Rheumatoid factor levels tended to increase with increasing age (p=0.066). Conclusion We report a detailed analysis of a large SLE under long term follow-up focusing on age at diagnosis related differences. Elderly patients differ from younger patients in clinical presentation. In vlSLE, female predominance is less pronounced; arthritis, anti-dsDNA antibodies and low C3 levels are less frequent. Concomitant SS and RA are more common. Our results confirm that older-onset SLE patients have lower disease activity. Nevertheless, these patients have the lowest survival rate. While very uncommon, SLE should not be excluded as a possible diagnosis in the elderly. Disclosure L. Viveiros: None. A. Neves: None. D.A. Isenberg: None.

Antiphospholipid syndrome in rural, remote, and First Nations peoples in the Top End of the Northern Territory, Australia

BackgroundThe Northern Territory of Australia has a high proportion of First Nations peoples living in remote communities and a high burden of chronic autoimmune diseases. The epidemiology and clinical outcomes of antiphospholipid syndrome (APS) in First Nations Australians are poorly characterized. ObjectivesTo determine the epidemiology, presenting features, and outcomes of patients with APS using an 18-year retrospective cohort of newly diagnosed patients presenting to Royal Darwin Hospital (2002-2020). MethodsPatients admitted to Royal Darwin Hospital with a new incident diagnosis of APS between January 2002 and December 2020 were identified and followed until December 2022, with data on baseline demographics, clinical and laboratory features, and overall survival extracted from electronic and paper medical records. ResultsFifty-three patients with APS were included, of whom 40 (75%) were First Nations and 46 (87%) were female. Thirty (75%) of First Nations patients with APS resided in very remote Australia vs 0 (0%) non–First Nations patients. Eighteen cases (34%) had primary APS, and 35 cases (66%) had secondary APS, most in association with lupus. Eight (15%) cases developed catastrophic APS (CAPS), all in First Nations patients. There were 13 deaths (of which 11 were among First Nations patients). Patients with CAPS had significantly shorter median overall survival (8.3 years from diagnosis), with median survival in non-CAPS patients not reached (P = .003). ConclusionThere is a high prevalence of APS in First Nations patients living in very remote Australia admitted for tertiary care in the tropical north of the Northern Territory, Australia. The rate of CAPS in First Nations patients was high, and CAPS was associated with significantly shorter survival. Larger prospective studies are required to inform improved models of care for First Nations and remote Australians living with APS.

Gender differences in SLE: report from a cohort of 417 Caucasian patients

BackgroundSLE is an autoimmune disease that predominantly affects women. As most epidemiological and interventional studies are on populations with a clear female prevalence, the influence of gender in disease course,...

Antiphospholipid antibodies in pulmonary embolism treated with direct oral anticoagulants: Prevalence data from unselected consecutive patients

BackgroundDirect oral anticoagulants (DOACs) are the first-choice treatment option for the prevention of the recurrence of venous thrombosis in patients with pulmonary embolism (PE); however, their effect in patients with antiphospholipid syndrome (APS) is challenged. Therefore, the prevalence of antiphospholipid autoantibodies in patients with PE is noteworthy. ObjectivesTo determine the prevalence of unselected patients presenting with PE who meet the criteria for APS based on elevated levels of anticardiolipin (aCL) and anti-β2-glycoprotein I (aβ2GPI) antibodies. MethodsConsecutive patients with PE, in whom DOACs were primarily initiated, were tested for aCL and aβ2GPI. If the levels were elevated, the tests were repeated after 12 weeks for APS diagnosis. Laboratory results and patient characteristics were retrospectively collected from a laboratory information system and electronic patient journal entries over a 2-year period. ResultsThe prevalence of APS based on consistently elevated levels of aCL or aβ2GPI was 3.7% (10 of 267 patients). Three patients were double positive. In 11 out of 21 patients (52%) with initially elevated values, the levels of the antibodies normalized after 12 weeks. The patient characteristics were largely similar in those with APS and those without APS; however, patients with APS tended to be older and more likely to receive antithrombotic treatment at the time of PE. ConclusionWe found a relatively low prevalence of APS based on aCL or aβ2GPI. The high rate of normalized levels of the antibodies after 12 weeks reaffirms the need for repeated tests for APS diagnosis. Older patients more frequently met the criteria for APS. Determining the effectiveness of DOACs in non–triple-positive patients with APS following venous thromboembolism is important to further determine the feasibility of unselected tests in patients with PE.

Ethnicity and Antiphospholipid Syndrome in Israel.

Antiphospholipid syndrome (APS) is an acquired coagulopathy associated with the presence of antiphospholipid antibodies. Whether ethnicity modulates APS clinical course is not known. The aim of our study was to assess the interplay of ethnicity and APS in Israel. We retrospectively evaluated the ethnic distribution of APS patients from 3 medical centers in Israel compared to the general population. Ethnic groups were defined according to the Israeli Bureau of Statistics as Ashkenazi (European), former Union of Soviet Socialist Republics (USSR), North African, Asian (West Asia, Greece, and Turkey), Israeli Arab individuals, and others. Our cohort included 382 patients. The prevalence of Ashkenazi and Asian ethnicities was more pronounced (33% versus 12.8% and 15.4% versus 7.7%, respectively; P < 0.001), while Israeli Arabs were less represented (5.2% versus 31.1%; P < 0.001) relative to their part in the general population. Arab patients were younger at presentation (mean ± SD 28 ± 10 years versus 34 ± 13 years; P < 0.001) and were more likely to present with venous thrombosis (50% versus 35%; P = 0.037) and to suffer from venous thrombotic recurrence (45% versus 16%; P < 0.001) compared to other ethnicities. Mortality was higher among patients of Asian ethnic origin (8.8% versus 1.1%; P = 0.005); intriguingly, this group experienced cardiovascular risk factors more often (i.e., dyslipidemia and hypertension). Ethnicity may affect the prevalence and/or natural course of APS, which is less prevalent and differs clinically in Israeli Arab patients, while mortality was linked with Asian ethnicity.

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.

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.

Antiphospholipid syndrome (APS): Clinical and immunoserological differences between primary and secondary APS in a Colombian cohort

IntroductionAntiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent thrombosis that can affect the arterial and venous circulation. ObjectivesTo analyze the immunological and pharmacological differences, as well as the clinical outcomes of a cohort of patients with primary antiphospholipid syndrome and secondary antiphospholipid syndrome. Materials and methodsA retrospective cohort study was conducted that included 352 records of patients diagnosed with APS and treated between 2014 and 2018. A description is presented of the sociodemographic, clinical, and immunological profile of the population. A bivariate analysis performed using the chi-squared test to determine differences between groups with primary APS and secondary APS, and finally a multivariate analysis to search for associations with thrombotic clinical outcomes in patients with APS. ResultsThe mean age was 42.4 ± 14 years, and 84.6% were females. Two-thirds (67.6%) of the patients had a diagnosis of primary APS, and 32.4% of secondary APS, of which 84% were associated with systemic lupus erythematosus (SLE). Among the thrombotic events, the most frequent were deep vein thrombosis (17.3%) and stroke (9.9%). Obstetric events were frequent, with a prevalence of 39.4% for miscarriages. No differences were found in the sociodemographic or immunoserological profile when comparing the group of primary vs. secondary APS. Thrombotic events were more frequent in the primary APS group, although only pulmonary embolism reached statistical significance. There were no differences between the two groups as regards obstetric events, such as miscarriages. Women were found to be 5 times more likely to have a stroke and 3 times more to have deep vein thrombosis. The anti-B2GPI type IgM increased the probability of presenting miscarriages about 3 times in women with APS. ConclusionThe study contains one of the largest Colombian cohorts with APS reported so far, and although it is both clinically and sociodemographically similar to other cohorts, there is a higher prevalence of primary APS. There was a lower frequency of thrombotic complications compared to other cohorts. Patients with primary APS had a tendency to develop thrombosis, as has already been reported in the literature.

Antiplatelet and anticoagulant agents for secondary prevention of stroke and other thromboembolic events in people with antiphospholipid syndrome.

Background Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial or venous thrombosis (or both) and/or pregnancy morbidity in association with the presence of antiphospholipid antibodies. The prevalence is estimated at 40 to 50 cases per 100,000 people. The most common sites of thrombosis are cerebral arteries and deep veins of the lower limbs. People with a definite APS diagnosis have an increased lifetime risk of recurrent thrombotic events. Objectives To assess the effects of antiplatelet or anticoagulant agents, or both, for the secondary prevention of recurrent thrombosis, particularly ischemic stroke, in people with antiphospholipid syndrome. Search methods We searched the Cochrane Stroke Group Trials Register (February 2017), CENTRAL (last search February 2017), MEDLINE (from 1948 to February 2017), Embase (from 1980 to February 2017), and several ongoing trials registers. We also checked the reference lists of included studies, systematic reviews, and practice guidelines, and we contacted experts in the field. Selection criteria We included randomized controlled trials (RCTs) that evaluated any anticoagulant or antiplatelet agent, or both, in the secondary prevention of thrombosis in people diagnosed with APS according to the criteria valid when the study took place. We did not include studies specifically addressing women with obstetrical APS. Data collection and analysis Pairs of review authors independently selected studies for inclusion, extracted data, and assessed the risk of bias for the included studies. We resolved any discrepancies through discussion or by consulting a third review author and, in addition, one review author checked all the extracted data. Main results We included five studies involving 419 randomized participants with APS. Only one study was at low risk of bias in all domains. One study was at low risk of bias in all domains for objective outcomes but not for quality of life (measured using the EQ-5D-5L questionnaire). We judged the other three studies to be at unclear or high risk of bias in three or more domains. The duration of intervention ranged from 180 days to a mean of 3.9 years. One study compared rivaroxaban (a novel oral anticoagulant: NOAC) with standard warfarin treatment and reported no thrombotic or major bleeding events, but it was not powered to detect such differences (low-quality evidence). Investigators reported similar rates of clinically relevant non-major bleeding (risk ratio (RR) 1.45, 95% confidence interval (CI) 0.25 to 8.33; moderate-quality evidence) and minor bleeding (RR 1.21, 95% CI 0.51 to 2.83) for participants receiving rivaroxaban and the standard vitamin K antagonists (VKA). This study also reported some small benefit with rivaroxaban over the standard VKA treatment in terms of quality of life health state measured at 180 days with the EQ-5D-5L 100 mm visual analogue scale (mean difference (MD) 7 mm, 95% CI 2.01 to 11.99; low-quality evidence) but not measured as health utility (MD 0.04, 95% CI −0.02 to 0.10 [on a scale from 0 to 1]). Two studies compared high dose VKA (warfarin) with moderate/standard intensity VKA and found no differences in the rates of any thrombotic events (RR 2.22, 95% CI 0.79 to 6.23) or major bleeding (RR 0.74, 95% CI 0.24 to 2.25) between the groups (low-quality evidence). Minor bleeding analyzed using the RR and any bleeding using the hazard ratio (HR) were more frequent in participants receiving high-intensity warfarin treatment compared to the standard-intensity therapy (RR 2.55, 95% CI 1.07 to 6.07; and HR 2.03, 95% CI 1.12 to 3.68; low-quality evidence). In one study, it was not possible to estimate the RR for stroke with a combination of VKA plus antiplatelet agent compared to a single antiplatelet agent, while for major bleeding, a single event occurred in the single antiplatelet agent group. In one study, comparing combined VKA plus antiplatelet agent with dual antiplatelet therapy, the RR of the risk of stroke over three years of observation was 5.00 (95% CI 0.26 to 98.0). In a single small study, the RR for stroke during one year of observation with a dual antiplatelet therapy compared to single antiplatelet drug was 0.14 (95% CI 0.01 to 2.60). Authors' conclusions There is not enough evidence for or against NOACs or for high-intensity VKA compared to the standard VKA therapy in the secondary prevention of thrombosis in people with APS. There is some evidence of harm for high-intensity VKA regarding minor and any bleeding. The evidence was also not sufficient to show benefit or harm for VKA plus antiplatelet agent or dual antiplatelet therapy compared to a single antiplatelet drug. Future studies should be adequately powered, with proper adherence to treatment, in order to evaluate the effects of anticoagulants, antiplatelets, or both, for secondary thrombosis prevention in APS. We have identified five ongoing trials mainly using NOACs in APS, so increasing experimental efforts are likely to yield additional evidence of clinical relevance in the near future.

Antiphospholipid Syndrome Is Still a Rare Disease—Estimated Prevalence in the Piedmont and Aosta Valley Regions of Northwest Italy: Comment on the Article by Duarte‐García et al

We read with interest the recent article by Duarte-García, A. et al. where they reported an estimated prevalence of the antiphospholipid syndrome (APS) of 50 cases per 100,000 population. APS is an autoimmune disorder characterized by thrombotic events, pregnancy morbidity, or both in the presence of persistent positivity for antiphospholipid antibodies (aPL). While APS is often held as the most common acquired thrombophilia, the global incidence and the prevalence of APS in the general population still need to be fully elucidated. Some reports estimate an incidence of 5 cases per 100,000 persons per year and a prevalence of 40-50 cases per 100.000 persons. Recently, several studies made further attempts to estimate the prevalence of aPL in different cohorts, such as young patients with stroke, patients with pregnancy morbidity, stroke, myocardial infarction, deep vein thrombosis, and patients with first unprovoked thrombosis.

P5359Pulmonary artery pressure is elevated three month after delivery in patients with preeclampsia and antiphospholipid syndrome

Abstract Introduction Preeclampsia (PE) is a multisystem disease affecting 2–8% of all pregnancies. Recently we found that the prevalence of antiphospholipid syndrome (APS) in PE patients at 13.9%. APS can be the cause of thromboembolic pulmonary hypertension. However, data on heart function and pulmonary pressure in PE and in PE with APS are sparse. Purpose We sought to investigate cardiac hemodynamics in this population. Method Between July 2016 and December 2018, we performed echocardiography in patients who had suffered from PE alone (n=102, age=32.6±4.8 y) or PE with APS (n=21, age=32.1±4.2 y) 3 months after delivery. APS was diagnosed according to the Sapporo criteria. Results The overall prevalence of APS was 17%. Right ventricular to right atrial pressure (RV/RA) gradient was significantly higher in PE + APS patients than in patients who had PE only (21.1±3.8 vs. 17.9±4.6mmHg, p=0.04, PE+APS vs. PE). The indexed volume of the left atrium (LA: 20.3±4.4 vs 23.5±5.5ml/m2, p=0.01, PE+APS vs. PE) and the birthweight of the newborn (1379.8±759.1 vs 1848.1±879.6.1g, p=0.02, PE+APS vs. PE) were lower in APS patients. Conclusion In patients with preeclampsia and APS, three month after delivery, pulmonary artery pressure was higher and the left atrium size smaller than in patients who had PE only. Conceivably this may reflects multiple subclinical clots in the pulmonary vessels in APS patients and may put these patients at an elevated risk of pulmonary hypertension later in life.

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.

THE PREVALENCE OF ANTIPHOSPHOLIPID SYNDROME IN WOMEN AFTER PREECLAMPSIA

Objective:Preeclampsia (PE) is a multisystem progressive disorder that affects 2–8% of all pregnancies. Severe and early PE (<34 weeks) is one of the clinical criteria of the obstetric antiphospholipid syndrome (APS). The aim of this study was to assess the prevalence of APS in women after PE.Design

Антифосфоліпідний синдром у дітей

The article presents modern data on the prevalence of antiphospholipid syndrome in children. The analysis of the clinical course of syndrome in children was provided and the laboratory criteria for the diagnosis of the disease are specified. Problems that arise in the management of children with antiphospholipid syndrome and possible ways of their solution are determined and discussed.

Management of antiphospholipid syndrome

Antiphospholipid syndrome, also known as ‘Hughes Syndrome’, is an autoimmune disease characterised by a set of clinical manifestations, almost all of which are direct or indirect sequelae of a hypercoagulable...

Aetiology of recurrent miscarriage and the role of adjuvant treatment in its management: a retrospective cohort review

We conducted a retrospective review into the role of commonly prescribed conventional adjuvant treatments in improving live birth rates after recurrent miscarriage (RM). Data from 301 couples attending the RM clinic in two Tertiary teaching hospitals were analysed with their live birth rate following a further pregnancy and a prevalence of conditions investigated in RM being the main outcomes measured. We found that 26% of women had explained RM and 74% had unexplained RM. Adjuvant versus conservative management did not improve the live birth rates in those with unexplained RM (68.4% vs. 76.6%, respectively; p = .28). The prevalence of anti-phospholipid syndrome, inherited thrombophilia, thyroid disease, parental karyotype abnormalities and structural uterine abnormalities were 7.4%, 4.5%, 6.6%, 2.9% and 6.6%, respectively. In conclusion, empirical adjuvant treatment for the management of women with unexplained RM does not appear to offer any benefit as they have a good prognosis with early pregnancy support alone.Impact statementWhat is already known on this subject? Does the adjuvant treatment in the management of unexplained recurrent miscarriage (RM) improve successful pregnancy outcomes? High-quality data regarding the management and outcomes of RM is very limited, with many clinicians prescribing adjuvant treatments for unexplained RM with very little good quality evidence of their benefit or risk.What do the results of this study add? We carried out a retrospective cohort study of all patients attending a recurrent miscarriage clinic over a two-year period at specialist clinics in two tertiary referral centres to evaluate the prevalence of associated diseases, the treatments given and the outcomes in subsequent pregnancies. This study will help clinicians counsel their patients about management options in RM and help them reassure their patients that the prognosis with conservative management alone is good. This will help to avoid any unnecessary use of adjuvant treatment and its associated risks and cost.What are the implications of these findings for clinical practice and/or further research? This study demonstrates that adjuvant treatments in unexplained RM have no significant benefit on future live birth rates. Despite this finding, high quality, prospective, randomised controlled trials looking at both adverse outcomes and benefits of adjuvant treatment in RM are needed.

Infodemiology of antiphospholipid syndrome: Merging informatics and epidemiology.

To investigate trends in Internet search volumes linked to Antiphospholipid Syndrome (APS), using Big Data monitoring and data mining. Based on the large amount of data generated by Google Trends and scientific search tools (SCOPUS, Medline/Pubmed, and ClinicalTrails.gov), we performed a longitudinal analysis based on the term "antiphospholipid" in a 5-year web-based research. Google Trends captured that APS-related digital interest was generally steady in the study period (Relative Search Volume [RSV] mean value 71.1±9.3% [95%CI 55.6-89.4], median 72.0), with no significant peak based on different seasons (e.g. winter vs. summer time). When comparing the APS-related digital interest with search volumes generated in the same time period for Inherited Thrombophilias (IT) and Systemic Lupus Erythematosus (SLE), we found a digital interest 35-times higher for APS than for IT (RSV mean value 71.1±9.3% [95%CI 55.6-89.4] vs. 2±3.2% [95%CI 0.7-7.4]). When compared to SLE, APS reached a similar RSV, showing a comparable digital interest (RSV mean value 71.1±9.3% [95%CI 55.6-89.4] vs. 87±11.8% [95%CI 60.7-107.9]). When adjusting for relative search volumes of Google Trends, we found a relative prevalence of search volumes of 35.5% in Europe, 12.3% in the United States, 11.5% in South America, 11.2% in Australia, 9.2% in Canada, 9.2% in Japan, and 5.1% in India. We observed an overall similar distribution of search volumes from Google Trends compared to results from Medline/Pubmed, SCOPUS, and ClinicalTrials.gov. In brief, the United States and Europe (mainly Italy, the United Kingdom, Spain, France, and Germany) presented the higher RSV. Similarly, these countries showed a higher number of research publications and on-going trials in the field of APS. In this study, we demonstrated that the interest in APS is not equally distributed globally. Thus, geopolitical differences might represent a challenge when attempting to estimate the prevalence of APS or designing worldwide investigations in APS. Combining the expanding framework of infodemiology with scientific networking collaborative efforts, such as AntiPhospholipid Syndrome Alliance For Clinical Trials and InternatiOnal Networking (APS ACTION), will help better define the syndrome in terms of prevalence, event occurrence ratios, and thrombosis risk assessment.

Study to assess the prevalence of antiphospholipid syndrome (APS) among women with adverse pregnancy outcome in a tertiary care centre

Study to assess the prevalence of antiphospholipid syndrome (APS) among women with adverse pregnancy outcome in a tertiary care centre

Relationship of complement activation route with clinical manifestations in Japanese patients with systemic lupus erythematosus: A retrospective observational study

Objectives. To assess the relationship between the complement activation route and clinical manifestations in systemic lupus erythematosus (SLE).Methods. Patients with SLE in whom complement activation occurred were divided into two groups: those in whom the complement system was mainly activated through the classical pathway (low serum C3 and C4 levels; CP group); and those in whom the complement system was solely activated through the alternative pathway (low serum C3 with normal C4 levels; AP group). Clinical manifestations were compared between the groups.Results. The CP group had higher frequencies of arthritis, serositis, and nephritis, and a higher prevalence of anti-DNA antibodies compared to the AP group (arthritis: 50.0% vs. 13.0%, p = 0.0014; serositis: 37.5% vs. 13.0%, p = 0.0257; nephritis: 63.6% vs. 21.7%, p = 0.0003; anti-DNA antibodies: 73.9% vs. 30.4%, p = 0.0001). In contrast, the AP group had a higher frequency of anti-phospholipid (anti-PL) antibodies and a higher prevalence of antiphospholipid syndrome (APS) (anti-PL antibodies: 70.6% vs. 37.3%, p = 0.0136; APS: 39.1% vs. 5.7%, p < 0.0001).Conclusions. Our results suggest that a different complement system mechanism may act in the pathogenesis of APS in patients with SLE.

Prevalence of the Antiphospholipid Syndrome and Its Effect on Survival in 679 Chinese Patients With Systemic Lupus Erythematosus

In this work we evaluate the prevalence of the antiphospholipid syndrome (APS) and its impact on survival in Chinese patients with systemic lupus erythematosus (SLE). We studied a prospective cohort of southern Chinese patients who fulfilled ≥4 American College of Rheumatology criteria for SLE. The cumulative rate of survival over time was calculated by the Kaplan-Meier method. APS was defined by the 2006 updated consensus criteria. We evaluated the prevalence and manifestations of APS, and compared the survival of patients with and without APS. We followed 679 patients with SLE (92% women; age of onset, 32.5 ± 14 yr) for 9.7 ± 7.3 years. Sixty-eight (10%) patients died and 33 (4.9%) patients were lost to follow-up. Forty-four (6.5%) patients met the criteria for APS, manifested by the following: ischemic stroke (55%), deep venous thrombosis (32%), obstetric morbidity (14%), cardiovascular events (9%), and peripheral vascular disease (9%). Nine (9/44 [20%]) APS patients died, which was more frequent than the non-APS patients (59/635 [9%]; p = 0.02). The cumulative mortality of patients with APS was 4.6% at 5 years, 7.8% at 10 years, and 22.2% at 15 years, which was not significantly higher than that of non-APS patients (5.4% at 5 years, 9.2% at 10 years, and 11.3% at 15 years; p = 0.14). However, if we considered only patients with APS caused by arterial thrombosis, the presence of APS was significantly associated with mortality (hazard ratio, 2.29; 95% confidence interval, 1.13–4.64; p = 0.02). We conclude that the presence of APS increases the mortality risk of Chinese patients with SLE, which is mainly contributed by arterial thrombotic events.Clinical significance: 1) APS is infrequent in southern Chinese patients with SLE compared to white patients. 2) Arterial thrombosis is a more common manifestation of APS than venous thrombosis in Chinese SLE patients. 3) APS related to arterial thrombosis is associated with increased mortality in Chinese patients with SLE.