-
Coronary Artery Disease Jun 2017Antiphospholipid antibody syndrome (APS) is a disorder associated with both arterial and venous thromboembolic disease, including acute myocardial infarction (AMI).... (Review)
Review
BACKGROUND
Antiphospholipid antibody syndrome (APS) is a disorder associated with both arterial and venous thromboembolic disease, including acute myocardial infarction (AMI). Given that management with anticoagulants is critical and differs from usual AMI care, identification of key discriminators of patients with AMI with APS is important.
METHODS
We performed an English-language systematic review of the literature of cases and case series of patients with AMI and APS from inception until 20 March 2016, collecting demographics, investigations, and outcomes.
RESULTS
Forty cases of AMI because of APS were identified from 27 articles. Patients were younger than typical AMI patients (41.10±13.61 years) and 45% were women. STelevation myocardial infarction was the presentation in 45% (18/40) of cases. The average platelet count was 130 000±138 912 c/mm in the 10 cases reporting it and partial thromboplastin time was elevated in all four reporting it. Coronary arteries were described as normal or with acute thrombosis in 75%. Three died during hospitalization and six had recurrence of myocardial infarction within 3 months after admission.
CONCLUSION
APS should be considered in young patients with AMI, especially if previous unprovoked thromboses, lower platelet counts, high partial thromboplastin times, and normal coronary arteries or coronary thromboses are identified.
Topics: Antiphospholipid Syndrome; Humans; Myocardial Infarction; Risk Factors
PubMed: 28225386
DOI: 10.1097/MCA.0000000000000476 -
International Journal of Molecular... Jun 2020The relationship between antiphospholipid antibodies (aPL) and autoimmune haemolytic anaemia (AIHA) has never been systematically addressed. The aim of this study is to... (Meta-Analysis)
Meta-Analysis Review
The relationship between antiphospholipid antibodies (aPL) and autoimmune haemolytic anaemia (AIHA) has never been systematically addressed. The aim of this study is to assess the link between aPL and AIHA in adult systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). This study performed an EMBASE/PubMed search from inception to June 2019 and meta-analysis using Peto's odds ratios. The pooled prevalence (PP) of IgG/IgM anticardiolipin (aCL) and lupus anticoagulant (LA) was greater in AIHA +ve than AIHA -ve patients (34.7% vs. 27.6%, = 0.03; 33.3% vs. 21.8%, < 0.0001; 20.9% vs. 8.3%, = 0.01). The PP of AIHA was greater in: (1) IgG and IgM aCL +ve than -ve patients (21.8% vs. 11.1%, = 0.001 and 18.7% vs. 6.3%, < 0.0001), (2) in SLE related APS than in primary APS patients (22.8% vs. 3.9% < 0.0001), (3) in APS +ve than APS -ve SLE patients (23.2% vs. 8.4%, = 0.01), and (4) in thrombotic APS than non-thrombotic APS/SLE patients (26.8% vs. 10%, = 0.03). The PP of IgG/IgM aCL and LA was greater in DAT +ve than DAT -ve patients (42.4% vs. 12.8%, < 0.0001; 26.2% vs. 12.8%, = 0.03 and 29.2% vs. 15.7%, = 0.004 respectively). It was found that AIHA prevalence is maximal in SLE with aPL/APS, low-moderate in SLE without aPL and minimal in PAPS. Moreover, AIHA is rightly included among the classification criteria for SLE but not for APS/aPL. The significance of an isolated DAT positivity remains unclear in this setting.
Topics: Anemia, Hemolytic, Autoimmune; Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Coombs Test; Humans; Lupus Erythematosus, Systemic; Thrombosis
PubMed: 32527000
DOI: 10.3390/ijms21114120 -
Lupus Science & Medicine Oct 2023Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterised by venous thrombosis (VT) or arterial thrombosis (AT) and/or pregnancy morbidity and the... (Meta-Analysis)
Meta-Analysis
Therapy with direct oral anticoagulants for secondary prevention of thromboembolic events in the antiphospholipid syndrome: a systematic review and meta-analysis of randomised trials.
OBJECTIVE
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterised by venous thrombosis (VT) or arterial thrombosis (AT) and/or pregnancy morbidity and the presence of antiphospholipid antibodies. Direct oral anticoagulants (DOACs) hold several advantages to vitamin K antagonists (VKAs) for prevention of thrombosis and we wish to evaluate DOACs compared with VKAs in secondary prevention of thromboembolic events in patients with APS.
METHODS
We conducted searches of the published literature using relevant data sources (MEDLINE, Embase and Cochrane CENTRAL), and of trial registers for unpublished data and ongoing trials. We included randomised trials examining individuals >18 years with APS classified according to the criteria valid when the trial was carried out. Randomised controlled trials had to examine any DOAC agent compared with any comparable drug. We tabulated all occurrences of events from all eligible randomised trials. Due to few events, ORs and 95% CIs were calculated using the Peto method.
RESULTS
5 randomised trials comprising 624 patients met the predefined eligibility criteria. The primary outcome measure was new thrombotic events, a composite endpoint of any VT or AT, during the VKA-controlled phase of treatment. According to the I inconsistency index, there was evidence of statistical heterogeneity across the studies (I=60%). Across trials, 29 and 10 thrombotic events were observed in 305 and 319 patients with APS treated with DOAC and VKA, respectively, corresponding to a combined Peto OR of 3.01 (95% CI 1.56 to 5.78, p=0.001). There was a significantly increased risk of AT while treated with DOACs compared with VKA (OR 5.5 (2.5, 12.1) p<0.0001), but no difference in the risk of VT (p=0.87). We found no significant difference in risk of bleeding.
CONCLUSIONS
DOACs were associated with a significant increase in the risk of a new thrombotic event, especially AT, favouring standard prophylaxis with warfarin.
PROSPERO REGISTRATION NUMBER
CRD42019126720.
Topics: Humans; Antiphospholipid Syndrome; Secondary Prevention; Lupus Erythematosus, Systemic; Anticoagulants; Thrombosis; Randomized Controlled Trials as Topic
PubMed: 37899090
DOI: 10.1136/lupus-2023-001018 -
The Malaysian Journal of Pathology Dec 2016Antiphospholipid syndrome (APS) is a multisystem disease that may present as venous or arterial thrombosis and/or pregnancy complications with the presence of... (Review)
Review
BACKGROUND
Antiphospholipid syndrome (APS) is a multisystem disease that may present as venous or arterial thrombosis and/or pregnancy complications with the presence of antiphospholipid antibodies. Until today, heterogeneity of pathogenic mechanism fits well with various clinical manifestations. Moreover, previous studies have indicated that genes are differentially expressed between normal and in the disease state. Hence, this study systematically searched the literature on human gene expression that was differentially expressed in Obstetric APS.
METHODOLOGY
Electronic search was performed until 31st March 2015 through PubMed and Embase databases; where the following Medical Subject Heading (MeSH) terms were used and they had been specified as the primary focus of the articles; gene, antiphospholipid, obstetric, and pregnancy in the title or abstract. From 502 studies retrieved from the search, only original publications that had performed gene expression analyses of human placental tissue that reported on differentially expressed gene in pregnancies with Obstetric APS were included. Two reviewers independently scrutinized the titles and the abstracts before examining the eligibility of studies that met the inclusion criteria. For each study; diagnostic criteria for APS, method for analysis, and the gene signature were extracted independently by two reviewers. The genes listed were further analysed with the DAVID and the KEGG pathways.
RESULTS
Three eligible gene expression studies involving obstetric APS, comprising the datasets on gene expression, were identified. All three studies showed a reduction in transcript expression on PRL, STAT5, TF, DAF, ABCA1, and HBEGF in Obstetric APS. The high enrichment score for functionality in DAVID had been positive regulation of cell proliferation. Meanwhile, pertaining to the KEGG pathway, two pathways were associated with some of the listed genes, which were ErBb signalling pathway and JAK-STAT signalling pathway.
CONCLUSION
Ultimately, studies on a genetic level have the potential to provide new insights into the regulation and to widen the basis for identification of changes in the mechanism of Obstetric APS.
Topics: Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Complications; Transcriptome
PubMed: 28028299
DOI: No ID Found -
American Journal of Obstetrics and... Nov 2023We performed a systematic review to evaluate the clinical presentation and maternal and fetal outcomes in pregnancies with early-onset HELLP (hemolysis, elevated liver... (Review)
Review
OBJECTIVE
We performed a systematic review to evaluate the clinical presentation and maternal and fetal outcomes in pregnancies with early-onset HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome.
DATA SOURCES
PubMed, Ovid MEDLINE, Scopus, CINAHL, Cochrane Library, and ClinicalTrials.gov were queried from inception through January 1, 2023 with the following terms: "HELLP syndrome," "HELLP," "hemolysis, elevated liver enzymes, low platelets," "hemolysis, elevated liver enzymes, low platelets syndrome," "pre-viable," "peri-viable," "previable," "periviable," "first trimester," "second trimester," "before 23 weeks," "<23 weeks," "<23 week gestation," and "before 23 weeks gestation." We also included an additional case from our institution.
STUDY ELIGIBILITY CRITERIA
Abstracts, unpublished studies, and review articles were excluded, yielding 46 studies that met our inclusion criteria.
METHODS
Two reviewers (N.S.I. and M.H.M.) performed the study selection and subsequent data extraction independently, after which the results were reviewed together. PRISMA guidelines were followed, and our study was registered at PROSPERO (CRD42021292692).
RESULTS
A total of 55 patients had 58 pregnancies complicated by early-onset HELLP syndrome, including 3 with recurrent HELLP. The most common presenting signs/symptoms were abdominal pain (35/45; 78%), hypertension (32/49; 65%), nausea/vomiting (16/45; 36%), headache (13/45; 29%), and edema (8/45; 18%). Lactate dehydrogenase ≥600 IU/L was observed in 21 of 31 (68%) cases, whereas liver enzyme abnormalities and thrombocytopenia were reported in 48 of 51 (94%) and 50 of 54 (93%) cases, respectively. Maternal complications were encountered in 25 of 56 (45%) cases. The most common complications were hepatic (13/56; 23%), central nervous system-related (11/56; 20%), and respiratory (11/56; 20%). In 36 of 57 (63%) cases, pregnancy was terminated. Of the 21 continued pregnancies, early fetal death (at <20 weeks' gestation) was reported in 10 (48%), stillbirth in 6 (28%), and neonatal demise in 2 (10%). Living neonates were reported in 3 of 21 (14%) cases, all delivered at 23 weeks. The perinatal mortality rate was 73% (8/11). One case (2%) reported maternal death. Antiphospholipid syndrome was diagnosed in 14 of 29 (48%) cases.
CONCLUSION
Early-onset HELLP syndrome presents with symptoms similar to those observed in later gestation. Maternal complications are life-threatening, with the most common complications being hepatic, central nervous system-related, and respiratory. Fetal outcomes are poor.
Topics: Infant, Newborn; Female; Pregnancy; Humans; HELLP Syndrome; Hemolysis; Pregnancy Trimester, Second; Thrombocytopenia; Gestational Age
PubMed: 37150281
DOI: 10.1016/j.ajog.2023.04.046 -
Autoimmunity Reviews May 2017Antiphospholipid antibodies (aPLs) namely anticardiolipin (aCL) antibody, anti-β2-glycoprotein I (β2GPI) antibody and lupus anticoagulant (LA) are autoantibodies... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Antiphospholipid antibodies (aPLs) namely anticardiolipin (aCL) antibody, anti-β2-glycoprotein I (β2GPI) antibody and lupus anticoagulant (LA) are autoantibodies produced against anionic phospholipids and proteins on plasma membranes. Migraine is a primary headache disorder which has growing evidences of autoimmune-mediated pathogenesis and previous studies suggested the presence of aPLs in migraine patients.
AIMS
The aim of this study was to evaluate the comorbid association between aPLs (aCL, anti-β2GPI and LA) and migraine compared to healthy controls.
METHODS
Studies were searched through PubMed, ISI Web of Science and Google Scholar databases without restricting the languages and year (up to October 2016) and were selected based on the inclusion criteria. Two authors independently extracted data from the included studies. All analyses were conducted by using random effects model to calculate the odds ratio (OR) and 95% confidence interval (CI). Quality assessment was carried out by using the modified Newcastle-Ottawa Scale (NOS). Publication bias was evaluated via visualization of funnel plots, Begg's and Egger's tests.
RESULTS
The database searches produced 1995 articles, 13 of which were selected (912 migraineurs and 822 healthy controls). 8.59%, 15.21% and 4.11% of the migraineurs exhibited aCL, anti-β2GPI and LA which was 4.83, 1.63 and 3.03 times higher, respectively, than healthy controls. A significant presence of aCL (OR: 3.55, 95% CI: 1.59-7.95; p=0.002) or anti-β2GPI antibodies (OR: 2.02, 95% CI: 1.20-3.42; p=0.008) was observed in migraine patients, however, LA was not significantly associated (OR: 2.02, 95% CI: 0.50-8.37; p=0.320). Majority of the studies (n=10 of 13) demonstrated NOS score of 7 or above and no significant publication bias was observed.
CONCLUSION
Migraine might be an autoimmune-associated neurologic disorder. The presence of aCL or anti-β2GPI antibodies was significant in migraine patients compared to healthy controls, suggesting an involvement of these autoantibodies in migraine attack.
Topics: Adolescent; Adult; Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Autoantibodies; Case-Control Studies; Child; Comorbidity; Female; Humans; Migraine Disorders; Prospective Studies; beta 2-Glycoprotein I
PubMed: 28279839
DOI: 10.1016/j.autrev.2017.03.005 -
Clinical Immunology (Orlando, Fla.) Nov 2023Antiphospholipid syndrome (APS) is a systemic autoimmune disease clinically associated with thrombotic and obstetric events. Additional manifestations have been...
Clinical features, risk factors, and outcomes of diffuse alveolar hemorrhage in antiphospholipid syndrome: A mixed-method approach combining a multicenter cohort with a systematic literature review.
BACKGROUND
Antiphospholipid syndrome (APS) is a systemic autoimmune disease clinically associated with thrombotic and obstetric events. Additional manifestations have been associated with APS, like diffuse alveolar hemorrhage (DAH). We aimed to summarize all the evidence available to describe the presenting clinical features, their prognostic factors, and short- and long-term outcomes.
METHODS
We performed a mixed-method approach combining a multicenter cohort with a systematic literature review (SLR) of patients with incident APS-associated DAH. We described their clinical features, treatments, prognostic factors, and outcomes (relapse, mortality, and requirement of mechanical ventilation [MV]). Kaplan-Meier methods were used to estimate relapse and mortality rates, and Cox and logistic regression models were used to assess the factors associated as appropriate.
RESULTS
We included 219 patients with incident APS-associated DAH (61 from Mayo Clinic and 158 from SLR). The median age was 39.5 years, 51% were female, 29% had systemic lupus erythematosus, and 34% presented with catastrophic APS (CAPS). 74% of patients had a history of thrombotic events, and 26% of women had a history of pregnancy morbidity; half of the patients had a history of thrombocytopenia, and a third had valvulopathy. Before DAH, 55% of the patients were anticoagulated. At DAH onset, 65% of patients presented hemoptysis. The relapse rate was 47% at six months and 52% at one year. Triple positivity (HR 4.22, 95% CI 1.14-15.59) was associated with relapse at six months. The estimated mortality at one and five years was 30.3% and 45.8%. Factors associated with mortality were severe thrombocytopenia (< 50 K/μL) (HR 3.10, 95% CI 1.39-6.92), valve vegetations (HR 3.22, 95% CI 1.14-9.07), CAPS (HR 3.80, 95% CI 1.84-7.87), and requirement of MV (HR 2.22, 95% CI 1.03-4.80). Forty-two percent of patients required MV on the incident DAH episode. Patients presenting with severe thrombocytopenia (OR 6.42, 95% CI 1.77-23.30) or CAPS (OR 4.30, 95% CI 1.65-11.16) were more likely to require MV.
CONCLUSION
APS-associated DAH is associated with high morbidity and mortality, particularly when presenting with triple positivity, thrombocytopenia, valvular involvement, and CAPS.
Topics: Humans; Female; Adult; Male; Antiphospholipid Syndrome; Hemorrhage; Lung Diseases; Lupus Erythematosus, Systemic; Leukopenia; Risk Factors; Recurrence; Thrombocytopenia; Retrospective Studies; Multicenter Studies as Topic
PubMed: 37722463
DOI: 10.1016/j.clim.2023.109775 -
General Hospital Psychiatry 2018In this systematic review, we reviewed the association between Antiphospholipid antibody syndrome (APS) and psychosis and focused on the prevalence, clinical... (Review)
Review
OBJECTIVE
In this systematic review, we reviewed the association between Antiphospholipid antibody syndrome (APS) and psychosis and focused on the prevalence, clinical presentation, immunologic and neurological workup, treatment options, and clinical outcomes.
METHODOLOGY
We performed this systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)'s guidelines. We searched eight databases for potential articles and manually searched references and relevant articles of included studies. We included all articles reporting psychotic symptoms in patients with APS. Article quality was assessed using an adapted version of the Cancer Council Australia tool for case-series.
RESULTS
We included 23 articles of 454 articles found. The mean patient age at presentation was 39years and most patients were women. Delusions and hallucinations were the common clinical manifestations of APS-associated psychosis. Findings on neuroimaging were attributed to APS-associated thrombosis in most cases. Most patients had a complete resolution of psychotic symptoms.
CONCLUSION
APS-associated psychosis is rare. Later age of onset for psychosis, sudden onset, female sex, and comorbid medical and psychiatric symptoms should raise the suspicions for the presence of APS. APS-associated psychosis may have a favorable prognosis. However, further studies need to validate this conclusion.
Topics: Antiphospholipid Syndrome; Female; Humans; Male; Psychotic Disorders
PubMed: 29241089
DOI: 10.1016/j.genhosppsych.2017.11.005 -
Rheumatology (Oxford, England) Dec 2021Cognitive dysfunction is common in patients with aPL (including primary APS or APS associated with SLE). Neuroimaging biomarkers may contribute to our understanding of...
OBJECTIVES
Cognitive dysfunction is common in patients with aPL (including primary APS or APS associated with SLE). Neuroimaging biomarkers may contribute to our understanding of mechanisms of cognitive dysfunction in these cohorts. This review aimed to investigate: (i) the prevalence of cognitive dysfunction in studies including neuroimaging biomarkers; and (ii) associations between cognition and neuroimaging biomarkers in patients with APS/aPL.
METHODS
We conducted a systematic search of electronic databases PubMed, Science Direct, Scopus and PsycINFO, and included studies with descriptions of neuroimaging findings, cognitive dysfunction or both, in patients with aPL positivity (LA, IgG and IgM aCL and anti-β2 glycoprotein-I antibodies).
RESULTS
Of 120 search results we included 20 eligible studies (6 APS, 4 SLE with APS/aPL and 10 NPSLE). We identified a medium risk of bias in 6/11 (54%) of cohort studies and 44% of case-control studies, as well as marked heterogeneity in cognitive assessment batteries, APS and aPL definitions, and neuroimaging modalities and protocols. The prevalence of cognitive dysfunction ranged between 11 and 60.5%. Structural MRI was the most common imaging modality, reporting cognitive dysfunction to be associated with white matter hyperintensities, ischaemic lesions and cortical atrophy (four with cerebral atrophy, two with white matter hyperintensities and two with cerebral infarcts).
CONCLUSION
Our findings confirm that cognitive impairment is commonly found in patients with aPL (including APS, SLE and NPSLE). The risk of bias, and heterogeneity in the cognitive and neuroimaging biomarkers reported does not allow for definitive conclusions.
Topics: Antiphospholipid Syndrome; Biomarkers; Cognition; Cognitive Dysfunction; Dementia; Humans; Neuroimaging; Prevalence
PubMed: 34003972
DOI: 10.1093/rheumatology/keab452 -
Journal of Clinical Medicine Jan 2023Hydroxychloroquine (HCQ) has been used in the treatment of systematic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), but its effect on lupus activity... (Review)
Review
Effect of Hydroxychloroquine on Lupus Activity, Preeclampsia and Intrauterine Growth Restriction in Pregnant Women with Systemic Lupus Erythematosus and/or Antiphospholipid Syndrome: A Systematic Review and Meta-Analysis.
BACKGROUND
Hydroxychloroquine (HCQ) has been used in the treatment of systematic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), but its effect on lupus activity during pregnancy, preeclampsia and intrauterine growth restriction (IUGR) remains unclear.
METHODS
PubMed, Embase and Cochrane databases were searched before 11 September 2022 for randomized clinical trials (RCT) or observational studies involving additional HCQ treatment and pregnant women diagnosed as having SLE and/or APS/positive antiphospholipid antibodies (aPLs). Risks of high lupus activity, preeclampsia and IUGR were explored.
RESULTS
One RCT and 13 cohort studies were included. A total of 1764 pregnancies were included in the pooled meta-analysis (709 in the HCQ group vs. 1055 in the control group). After the additional use of HCQ, the risk of high lupus activity decreased (RR: 0.74, 95% CI: 0.57-0.97, = 0.03). For preeclampsia, the total incidence decreased (RR: 0.54, 95% CI: 0.37-0.78, = 0.001). The subgroup analysis showed statistical significance in the SLE subgroup (RR: 0.51, 95% CI: 0.34-0.78, = 0.002) but not in the APS/aPLs subgroup (RR: 0.66, 95% CI: 0.29-1.54, = 0.34). For IUGR, the decrease in incidence was not statistically significant (RR: 0.80, 95% CI: 0.47-1.35, = 0.46), neither in the SLE subgroup (RR: 0.74, 95% CI: 0.40-1.36, = 0.33) nor in the APS/aPLs subgroup (RR: 1.26, 95% CI: 0.34-4.61, = 0.73).
CONCLUSION
The additional use of HCQ may decrease the risk of high lupus activity during pregnancy and the incidence of preeclampsia for SLE patients, but the results do not support that using HCQ decreases the incidence of preeclampsia for APS/aPLs patients or reduces IUGR risk for SLE and/or APS/aPLs patients.
PubMed: 36675415
DOI: 10.3390/jcm12020485