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American Journal of Nephrology 2009Antiphospholipid antibodies are a heterogeneous group of autoantibodies associated with the hypercoagulable state affecting all vascular districts with thrombosis named... (Review)
Review
Antiphospholipid antibodies are a heterogeneous group of autoantibodies associated with the hypercoagulable state affecting all vascular districts with thrombosis named antiphospholipid syndrome (APS). APS is an autoimmune disease with multifactorial etiology that includes cellular, molecular, genetic and pathogenic mechanisms. The APS clinical features are a combination of arterial and/or venous thrombosis, hematological events, recurrent fetal losses, neurological disorders and intra-abdominal manifestations. The renal involvement is associated with both primary and secondary APS. Clinical features include hypertension, renal artery stenosis, thrombotic microangiopathy and other histological manifestations of the nephropathy (APSN), venous renal thrombosis, APSN in the course of systemic lupus erythematosus and renal failure. APSN is an independent risk factor that should be included in the classification criteria for definite APS with characteristic clinical and histological features.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Humans; Kidney; Kidney Diseases; Risk Factors
PubMed: 19713697
DOI: 10.1159/000235941 -
Frontiers in Immunology 2021Antiphospholipid syndrome (APS) is a multisystem disorder characterized by thrombosis and/or recurrent fetal loss. This clinical phenotype heterogeneity may result in...
Antiphospholipid syndrome (APS) is a multisystem disorder characterized by thrombosis and/or recurrent fetal loss. This clinical phenotype heterogeneity may result in differences in response to treatment and prognosis. In this study, we aimed to identify primary thrombotic APS (TAPS) from primary obstetric APS (OAPS) using urine proteomics as a non-invasive method. Only patients with primary APS were enrolled in this study from 2016 to 2018 at a single clinical center in Shanghai. Urine samples from 15 patients with TAPS, 9 patients with OAPS, and 15 healthy controls (HCs) were collected and analyzed using isobaric tags for relative and absolute quantification (iTRAQ) labeling combined with liquid chromatography-tandem mass spectrometry analysis to identify differentially expressed proteins. Cluster analysis of urine proteomics identified differentiated proteins among the TAPS, OAPS, and HC groups. Urinary proteins were enriched in cytokine and cytokine receptor pathways. Representative secreted cytokines screened out (fold change >1.20, or <0.83, <0.05) in these differentiated proteins were measured by enzyme-linked immunosorbent assay in a validation cohort. The results showed that the levels of C-X-C motif chemokine ligand 12 (CXCL12) were higher in the urine of patients with TAPS than in those with OAPS (=0.035), while the levels of platelet-derived growth factor subunit B (PDGFB) were lower in patients with TAPS than in those with OAPS (=0.041). In addition, correlation analysis showed that CXCL12 levels were positively correlated with immunoglobulin G anti-β2-glycoprotein I antibody (=0.617, =0.016). Our results demonstrated that urinary CXCL12 and PDGFB might serve as potential non-invasive markers to differentiate primary TAPS from primary OAPS.
Topics: Adult; Antiphospholipid Syndrome; Biomarkers; Female; Humans; Middle Aged; Pregnancy; Pregnancy Complications; Proteomics; Urinalysis
PubMed: 34489952
DOI: 10.3389/fimmu.2021.702425 -
Journal of Immunology Research 2014To evaluate the clinical associations between rheumatic fever and antiphospholipid syndrome and the impact of coexistence of these two diseases in an individual. (Review)
Review
OBJECTIVE
To evaluate the clinical associations between rheumatic fever and antiphospholipid syndrome and the impact of coexistence of these two diseases in an individual.
METHODS
Systematic review in electronics databases, regarding the period from 1983 to 2012. The keywords: "Rheumatic Fever," "Antiphospholipid Syndrome," and "Antiphospholipid Antibody Syndrome" are used.
RESULTS
were identified 11 cases described in the literature about the association of rheumatic fever and antiphospholipid syndrome. Clinical presentation of rheumatic fever was characterized by the predominance of carditis (11/11) and chorea (7/11). Regarding the manifestations of APS, the stroke was observed in 7/11 (63.6%), with one of them having probable embolic origin.
CONCLUSION
The present study brings the information that the association between APS and RF is quite rare, however, is of great clinical importance. Doctors who deal with the RF should include in their differential diagnosis the APS, especially in the presence of stroke in patients with RF and whose echocardiogram does not show intracavitary thrombi.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Chorea; Diagnosis, Differential; Humans; Myocarditis; Rheumatic Fever; Stroke
PubMed: 24860836
DOI: 10.1155/2014/614591 -
Journal of Thrombosis and Haemostasis :... Jun 2018The direct oral anticoagulants (DOACs) are therapeutic alternatives to warfarin and other vitamin K antagonists (VKAs), and constitute the standard of care for many... (Review)
Review
The direct oral anticoagulants (DOACs) are therapeutic alternatives to warfarin and other vitamin K antagonists (VKAs), and constitute the standard of care for many indications. VKAs constitute the conventional therapy for the treatment and secondary thromboprophylaxis of thrombotic antiphospholipid syndrome (APS), but are often problematic, owing to the variable sensitivity of thromboplastins to lupus anticoagulant. Thus, the International Normalized Ratio may not accurately reflect anticoagulation intensity, or be clinically effective. Definition of the current role of DOACs in the treatment of APS is based on limited clinical trial data and information from other sources, including manufacturers' data, case series or cohort studies, and expert consensus. The Rivaroxaban in Antiphospholipid Syndrome (RAPS) randomized controlled trial (RCT), which had a laboratory surrogate primary outcome measure, suggests that rivaroxaban has the potential to be an effective and convenient alternative to warfarin in thrombotic APS patients with a single venous thromboembolism event requiring standard-intensity anticoagulation. However, further studies, in particular to provide better long-term efficacy and safety data, are needed before it can be widely recommended. APS patients are clinically heterogeneous, with the risk of recurrent thrombosis and the intensity of anticoagulation being influenced by their clinical phenotype and risk profile. DOAC trials involving homogeneous thrombotic APS populations, with the antiphospholipid antibody status well defined, will help to optimize the appropriate treatment in APS patient subgroups. Ongoing and emerging DOAC RCTs should provide further information to guide the use of DOACs in APS patients. Optimal identification of APS patients is a key step in working towards improved therapeutic strategies in these individuals.
Topics: Administration, Oral; Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Biomarkers; Blood Coagulation; Clinical Decision-Making; Evidence-Based Medicine; Hemorrhage; Humans; Risk Assessment; Risk Factors; Thrombosis; Treatment Outcome
PubMed: 29624847
DOI: 10.1111/jth.14017 -
Frontiers in Immunology 2019Antiphospholipid syndrome (APS) is a multisystem autoimmune disease most commonly associated with recurrent arterial and venous thromboembolism and recurrent fetal loss.... (Review)
Review
Antiphospholipid syndrome (APS) is a multisystem autoimmune disease most commonly associated with recurrent arterial and venous thromboembolism and recurrent fetal loss. Other possible antiphospholipid antibody (aPL)-related clinical manifestations include cardiac involvement. The heart can be involved through immune mediated and /or thrombotic mechanisms. Mortality due to cardiovascular problems is elevated in APS. However, the cardiovascular risk in patients with primary APS (PAPS) compared with lupus-related APS is yet to be established. Cardiac symptoms of APS include valve abnormalities (thickening and vegetations), coronary artery disease (CAD), myocardial dysfunction, pulmonary hypertension, and intracardiac thrombi. Heart valve lesions are the most common cardiac manifestation, observed in approximately one third of PAPS patients and usually do not cause hemodynamic significance. Deposits of immunoglobulins including anticardiolipin (aCL), and of complement components, are commonly observed in affected heart valves from these patients. This suggests that an inflammatory process is initiated by aPL deposition, eventually resulting in the formation of valvular lesion. aPL may have a direct role in the atherosclerotic process via induction of endothelial activation. Multiple traditional and autoimmune-inflammatory risk factors are involved in triggering an expedited atherosclerotic arterial disease evident in APS. It is imperative to increase the efforts in early diagnosis, control of risk factors and close follow-up, in the attempt to minimize cardiovascular risk in APS. Clinicians should bear in mind that a multidisciplinary therapeutic approach is of paramount importance in these patients. This article reviews the cardiac detriments of APS, including treatment recommendations for each cardiac complication.
Topics: Antiphospholipid Syndrome; Heart Diseases; Humans
PubMed: 31134062
DOI: 10.3389/fimmu.2019.00941 -
Medizinische Klinik (Munich, Germany :... Feb 1999Antiphospholipid antibodies comprise a family of auto-antibodies mainly characterized by the presence of the lupus anticoagulant (LA) and anticardiolipin antibodies... (Review)
Review
BACKGROUND
Antiphospholipid antibodies comprise a family of auto-antibodies mainly characterized by the presence of the lupus anticoagulant (LA) and anticardiolipin antibodies (ACA).
CLINICAL APPEARANCE
The antiphospholipid antibody syndrome is defined by the appearance of frequent thromboses, repeated fetal losses and thrombocytopenia. Other clinical manifestations associated with APA include migraine, chorea, hemolytic anemia, heart valve disease, Budd-Chiari syndrome, perpetual pancreatitic episodes, intestinal infarctions, malignant hypertension, livedo reticularis, pre-eclampsia, fetal growth retardation or catastrophic antiphospholipid syndrome. LA and ACA occur in a variety of clinical conditions (secondary antiphospholipid antibody syndrome, SAPS), including other autoimmune disorders, infectious diseases, neoplastic disorders, in association with the use of certain drugs or in otherwise healthy individuals (primary antiphospholipid antibody syndrome, PAPS).
TREATMENT
Patients with thrombosis associated with APA should receive long-term anticoagulation therapy, whereas treatment of asymptomatic patients seems to be not indicated, because only approximately 10% of patients with APA may develop thrombotic complications. In patients with PAPS there is no evidence that the prophylactic administration of immunosuppressive drugs will prevent thromboembolic events.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Diagnosis, Differential; Humans
PubMed: 10194954
DOI: 10.1007/BF03044707 -
Lupus May 2014There is growing evidence that the commensal bacteria in the gastrointestinal tract (the gut microbiota) influence the development of autoimmunity in rodent models.... (Review)
Review
There is growing evidence that the commensal bacteria in the gastrointestinal tract (the gut microbiota) influence the development of autoimmunity in rodent models. Since humans have co-evolved with commensals for millennia, it is likely that people, who are genetically predisposed to autoimmunity, harbor gut microbial communities that similarly influence the onset and/or severity of disease. Beyond the current efforts to identify such disease-promoting or -preventing commensals ("pathobionts" or "symbionts"), it will be important to determine what factors modulate them. Dietary changes are known to affect both the composition and function of the gut microbial communities, which in turn can alter the innate and adaptive immune system. In this review, we focus on the relationships between diet, microbiota, and autoimmune diseases. We hypothesize that the beneficial and life-prolonging effects of caloric restriction on a variety of autoimmune models including lupus might partly be mediated by its effects on the gut microbiome and associated virome, the collection of all viruses in the gut. We give recent examples of the immunomodulatory potential of select gut commensals and their products or diet-derived metabolites in murine models of arthritis, multiple sclerosis, and type 1 diabetes. Lastly, we summarize the published phenotypes of germ-free mouse models of lupus and speculate on any role of the diet-sensitive microbiome and virome in systemic lupus and the related antiphospholipid syndrome.
Topics: Animals; Antiphospholipid Syndrome; Autoimmunity; Caloric Restriction; Diet; Disease Models, Animal; Gastrointestinal Tract; Host-Pathogen Interactions; Humans; Lupus Erythematosus, Systemic; Mice; Microbiota; Risk Assessment; Risk Factors
PubMed: 24763536
DOI: 10.1177/0961203313501401 -
Journal of Thrombosis and Haemostasis :... Aug 2005Antiphospholipid syndrome is a distinct disorder with the clinical features of recurrent thrombosis in the venous or arterial circulation and fetal losses. Its... (Review)
Review
Antiphospholipid syndrome is a distinct disorder with the clinical features of recurrent thrombosis in the venous or arterial circulation and fetal losses. Its serological marker is the presence of antiphospholipid antibodies in the blood of these patients. The relation between the presence of antibodies against anionic phospholipids and thromboembolic complications is well established over the last 25 years but the pathophysiology of the syndrome is largely unclear. Even after all these years, there is a persisting debate about the specificity and sensitivity of the assays for the detection of antiphospholipid antibodies. We now accept that antibodies to beta2-glycoprotein I rather than to anionic phospholipids are the major pathological antibodies, although there is no clear consensus on how the presence of these antibodies correlates with the different clinical manifestations of the syndrome. In this review, we discuss the current methods of detection of the antibodies and our insight into the pathobiology of the syndrome. We propose a mechanism for describing how the presence of anti-beta2-glycoprotein I antibodies relates to the different clinical manifestations observed.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Blood Platelets; Crystallography, X-Ray; Enzyme-Linked Immunosorbent Assay; Female; Glycoproteins; Humans; MAP Kinase Signaling System; Models, Biological; Models, Molecular; Pregnancy; Pregnancy Complications, Hematologic; beta 2-Glycoprotein I
PubMed: 16102052
DOI: 10.1111/j.1538-7836.2005.01359.x -
Frontiers in Immunology 2022Patients with laboratory or clinical manifestations suggestive of antiphospholipid syndrome (APS) but not fulfilling the classification criteria constitute a clinical...
OBJECTIVES
Patients with laboratory or clinical manifestations suggestive of antiphospholipid syndrome (APS) but not fulfilling the classification criteria constitute a clinical challenge. This study aims to compare non-criteria APS (NC-APS) with definite APS in terms of clinical manifestations, therapies, and outcomes.
METHODS
A systematic review of observational studies comparing definite and NC-APS was performed searching four electronic databases. Data on clinical manifestations, therapies and clinical outcomes was extracted.
RESULTS
Sixteen studies, assessing a total of 3,798 participants, were included. Seven out of 10 studies found no significant difference in the prevalence of arterial or venous thrombosis between definite and NC-APS, with two studies on seronegative APS also finding no difference in thrombosis recurrence. Seven out of 12 studies found no significant difference in the prevalence of obstetric manifestations between groups, with the remaining exhibiting conflicting results. In 9 studies comparing treatment frequency in obstetric patients, all but one described similar treatment frequency, with the percentage of NC-APS treated during pregnancy ranging from 26% to 100%. In 10 studies comparing pregnancy outcomes of NC-APS versus definite APS, 7 found similar successful pregnancies/live births. Additionally, 5 studies described improvement of live births in both groups with treatment, with three signalling aspirin monotherapy as efficacious as combination therapy in NC-APS.
CONCLUSION
This review hints at an absence of marked differences in most evaluated parameters between definite and NC-APS, emphasizing the value of a more active follow-up of these patients. The low-quality available evidence highlights the need for well-defined NC-APS populations in future studies.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero, identifier CRD42020210674.
Topics: Antiphospholipid Syndrome; Aspirin; Female; Humans; Pregnancy; Pregnancy Outcome; Thrombosis; Venous Thrombosis
PubMed: 36059460
DOI: 10.3389/fimmu.2022.967178 -
The Journal of Extra-corporeal... Mar 2021Antiphospholipid syndrome (APS) is an acquired autoimmune condition characterized by the presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin...
Antiphospholipid syndrome (APS) is an acquired autoimmune condition characterized by the presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibody, and anti-β glycoprotein-I antibody) which leads to clinical thrombosis via a multifactorial mechanism of action. Despite the propensity to form clot in vivo, these antibodies interfere with the assembly of the prothrombinase complex on phospholipids in in vitro assays, leading to prolongation of activated clotting time and activated partial thromboplastin time. This disconnect between what occurs in vivo and in vitro makes monitoring anticoagulation during cardiac surgery particularly complex. We present a patient with APS undergoing coronary artery bypass grafting with cardiopulmonary bypass. We delineate our strategy for managing anticoagulation in the presence of this syndrome using the Hepcon Hemostasis Management System Plus (Medtronic, Inc. Minneapolis, MN) device by targeting whole blood heparin concentration to monitor anticoagulation.
Topics: Anticoagulants; Antiphospholipid Syndrome; Blood Coagulation; Cardiopulmonary Bypass; Coronary Artery Bypass; Humans
PubMed: 33814605
DOI: 10.1182/ject-2000040