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Ugeskrift For Laeger May 2024Individuals with antiphospholipid syndrome (APS) have antibodies directed against phospholipid-binding proteins (aPL). The condition is most associated with an increased... (Review)
Review
Individuals with antiphospholipid syndrome (APS) have antibodies directed against phospholipid-binding proteins (aPL). The condition is most associated with an increased risk of thromboembolism and obstetric complications. The 2023 classification criteria for APS include six clinical domains (venous thromboembolism, arterial thrombosis, microvascular events, obstetric events, cardiac valve, thrombocytopaenia) and two laboratory domains (lupus anticoagulant, and anti-cardiolipin or anti-β2-glycoprotein-I antibodies). Diagnosis and treatment of APS are specialist tasks and are summarised in this review.
Topics: Antiphospholipid Syndrome; Humans; Antibodies, Antiphospholipid; Pregnancy; Female; Anticoagulants; Thrombosis
PubMed: 38847311
DOI: 10.61409/V11230715 -
Frontiers in Immunology 2023The presence of antiphospholipid antibodies (aPLs) plays a pivotal role in the pathogenesis of antiphospholipid antibody syndrome (APS). This study aimed to examine the...
BACKGROUND
The presence of antiphospholipid antibodies (aPLs) plays a pivotal role in the pathogenesis of antiphospholipid antibody syndrome (APS). This study aimed to examine the diagnostic value of a set of non-criteria aPLs and their relevance with APS-related criteria and extra-criteria manifestations.
METHODS
From a prospectively constructed database, consecutive APS patients consisting of 114 primary APS (PAPS group), 54 with APS secondary to SLE (SAPS group), 9 seronegative APS (SNAPS), as well as 209 patients with systemic lupus erythematosus (SLE) and 88 healthy controls were included in this study. Levels of criteria aPLs, baseline information, and APS-related criteria and extra-criteria features were extracted from the database. Serum levels of non-criteria aPLs including aPC IgG/IgM, aPI IgG/IgM, aPE IgG/IgM/IgA, aPG IgG/IgM/IgA, anti-phosphatidic acid (aPA) IgG/IgM, aSM IgG/IgM, and aPS/PT IgG/IgM were analyzed with AESKULISA® ELISA Test Kits.
RESULTS
The addition of aPC IgG/M, aPI IgG/M, aPE IgG/M/A, aSM IgG/M, and aPA IgG/M to aCL or aβ2GPI IgG/M could significantly increase diagnostic sensitivity and accuracy. A significant difference between PAPS or SAPS and HC was presented in all non-criteria aPLs except for aSM IgM and aPG IgA. Eight out of nine SNAPS patients were positive for at least 1 aPL. Pregnancy morbidity was associated with aSM IgM (r = 0.22) and aSM IgG (r = 0.15). Pre-eclampsia or premature birth was associated with aSM IgG (r = 0.16), aPI IgG (r = 0.22), aPC IgG (r = 0.16), and aPG IgG (r = 0.18). Stroke was associated with aPI IgG (r = 0.2). The clinical association was also observed in DVT with aPS/PT IgG (r = 0.17). Valve lesion was positively associated with aSM IgM (Fisher test p = 0.039), APS nephropathy was associated with aPC IgG (OR 3.797), and livedo reticularis was associated with aPE IgM (OR 15.391).
CONCLUSION
Additional detection of non-criteria aPLs including aPC IgG/M, aPE IgG/M/A, aPI IgG/M, aSM IgG/M, and aPA IgG/M could assist in APS diagnosis. The positivity of certain aPLs was statistically associated with both criteria and extra-criteria APS clinical manifestations.
Topics: Female; Humans; Pregnancy; Antibodies, Antiphospholipid; Antiphospholipid Syndrome; East Asian People; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Lupus Erythematosus, Systemic; Penicillamine; Prevalence
PubMed: 37122726
DOI: 10.3389/fimmu.2023.1107510 -
Lupus Oct 2018
Topics: Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Complications, Cardiovascular; Thrombosis
PubMed: 30452325
DOI: 10.1177/0961203318801686 -
Paediatric Drugs Jan 2022Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical... (Review)
Review
Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical manifestations. APS in children and adolescents typically presents with large-vessel thrombosis, thrombotic microangiopathy, and, rarely, obstetric morbidity. Non-thrombotic clinical manifestations are frequently seen in pediatric APS and may be present even before the vascular thrombotic events occur. We review insights into the pathogenesis of APS and discuss potential targets for therapy. The identification of multiple immunologic abnormalities in patients with APS reveals molecular targets for current or future treatment. Management strategies, especially for APS in adolescents, require screening for additional prothrombotic risk factors and consideration of counseling regarding contraceptive strategies, lifestyle recommendations, treatment adherence, and mental health issues associated with this autoimmune thrombophilia. The main goal of therapy in pediatric APS is the prevention of thrombosis. The management of acute thrombosis events in children and adolescents is the same as for primary APS, which involves isolated occurrences, and secondary APS, which is seen in association with another autoimmune disease, e.g., systemic lupus erythematosus. A pediatric hematologist should be consulted so other differential thrombophilic conditions can be eliminated. Therapy includes unfractionated heparin or low-molecular-weight heparin followed by vitamin K antagonists. Treatment of catastrophic APS involves triple therapy (anticoagulation, intravenous corticosteroid pulse therapy, and plasma exchange) and may include intravenous immunoglobulin for children and adolescents with this condition. New drugs such as eculizumab and sirolimus seem to be promising drugs for APS.
Topics: Adolescent; Anticoagulants; Antiphospholipid Syndrome; Child; Female; Heparin; Humans; Lupus Erythematosus, Systemic; Pregnancy; Thrombosis
PubMed: 34904182
DOI: 10.1007/s40272-021-00484-w -
The Journal of Investigative Dermatology Jan 1993The antiphospholipid antibodies (aPL), namely, the lupus anticoagulant and the anticardiolipin antibodies, are a family of autoantibodies directed predominantly against... (Review)
Review
The antiphospholipid antibodies (aPL), namely, the lupus anticoagulant and the anticardiolipin antibodies, are a family of autoantibodies directed predominantly against negatively charged phospholipids. Many studies have confirmed that patients with these antibodies are prone to repeated episodes of thrombosis, fetal losses, and thrombocytopenia. The association of aPL with these clinical events has been termed the antiphospholipid syndrome. Several skin lesions have been found in patients with this syndrome, including livedo reticularis, livedoid vasculitis, thrombophlebitis, cutaneous infarctions and gangrene of digits, ulcerations, lesions resembling vasculitis (nodules, macules), cutaneous necrosis/infarctions, subungual splinter hemorrhages, and, less commonly, discoid lupus and Degos' disease (malignant atrophic papulosis). In this article, we review the main immunologic and clinical aspects of this syndrome with special emphasis on the dermatologic features.
Topics: Antiphospholipid Syndrome; Catastrophic Illness; Humans; Prevalence; Skin Diseases
PubMed: 8423386
DOI: 10.1111/1523-1747.ep12355193 -
Autoimmunity Reviews Aug 2018Primary antiphospholipid syndrome (PAPS) and antiphospholipid syndrome associated to lupus (SAPS) have several overlapping characteristics. As systemic manifestations... (Review)
Review
Primary antiphospholipid syndrome (PAPS) and antiphospholipid syndrome associated to lupus (SAPS) have several overlapping characteristics. As systemic manifestations are also reported in patients with PAPS, and as a subgroup of PAPS patients could evaluate to a SAPS, the differentiation between the two types of APS could be performed based on the clinical experience of the medical teams and is related to a variety of clinical, biological, histological and genetic features. Several data are available in the literature with respect to the identification of distinctive features between these two entities. However, there are some limitation in the interpretation of results issued from studies performed prior to updated Sydney criteria. Based on recent data, a certain number of features more frequent in one type of APS as compared to the other could be distinguished. The major differentiation between these two entities is genetical. New genetic data allowing the identification of specific subgroups of APS are ongoing.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Humans; Lupus Erythematosus, Systemic
PubMed: 29885541
DOI: 10.1016/j.autrev.2018.01.027 -
RMD Open Mar 2023Antiphospholipid syndrome (APS) is defined by the association of thromboembolic and/or obstetrical clinical manifestations and the presence of antiphospholipid...
OBJECTIVE
Antiphospholipid syndrome (APS) is defined by the association of thromboembolic and/or obstetrical clinical manifestations and the presence of antiphospholipid antibodies. The objective of our study was to evaluate the impact of the triple-positive profile in a cohort of 204 APS patients.
METHODS
We conducted a retrospective study, including patients with primary or secondary APS, meeting the Sydney criteria with at least one thrombotic and/or obstetrical complication. Clinical characteristics and the risk of relapse (defined by the occurrence of a new thrombotic event and/or a new adverse obstetrical event) between triple-positive and non-triple-positive APS patients were compared.
RESULTS
204 patients were included in our study, 68 were triple-positive and 136 were single or double positive. 122 patients (59.8%) had primary APS. 67 patients (32.8%) had obstetrical APS, with a higher rate among triple-positive patients (45.6% vs 26.5%, p=0.010), and 170 patients (83.3%) had thrombotic APS, without difference between triple-positive and others. Thrombotic events were more often venous (56.4%) than arterial (37.7%). Triple-positive patients had more placental complications than others (17.6% vs 2.9%, p=0.001) and more non-criteria events (48.5% vs 25.7%, p=0.002). Among non-criteria events, there was a higher frequency of Sneddon syndrome in triple-positive patients (7.4% vs 0.7%, p=0.028). The relapse rate was higher in triple-positive patients than in others (63.2% vs 39,7%, p=0002). In multivariate analysis, the triple-positive profile was associated with a higher risk of relapse (HR 1.63; 95% CI 1.04 to 2.55; p=0.031).
CONCLUSION
The triple-positivity is associated with a higher risk of relapse and obstetrical complications.
Topics: Humans; Female; Pregnancy; Antiphospholipid Syndrome; Retrospective Studies; Placenta; Antibodies, Antiphospholipid; Prognosis; Thrombosis
PubMed: 37001919
DOI: 10.1136/rmdopen-2022-002534 -
Polskie Archiwum Medycyny Wewnetrznej 2013Obstetrical antiphospholipid syndrome (APS) is defined by obstetrical complications and the presence of antiphospholipid antibodies (aPL). Although the incidence of APS... (Review)
Review
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.
Topics: Abortion, Habitual; Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Complications
PubMed: 24382555
DOI: 10.20452/pamw.2025 -
Current Opinion in Rheumatology May 2019Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as... (Review)
Review
PURPOSE OF REVIEW
Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding - and persistent knowledge gaps - through clinically oriented questions and answers.
RECENT FINDINGS
A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis). Although the pathophysiology underlying thrombocytopenia in APS has yet to be definitively revealed, mechanisms that play a role (at least in subsets of patients) include: immune thrombocytopenic purpura/ITP-like autoantibodies against platelet glycoproteins; antiphospholipid antibody (aPL)-mediated platelet activation and consumption; and potentially life threatening thrombotic microangiopathy. Although thrombocytopenia is often 'mild' in APS (and therefore, may not require specific therapy), there are causes of acute-onset thrombocytopenia that mandate emergent work-up and treatment. When APS-related thrombocytopenia does require therapy, the approach must be individualized (requiring an understanding of pathophysiology in the particular APS patient). For patients with ITP-like disease, rituximab is emerging as a popular approach to treatment; in contrast, there are hints that thrombopoietin mimetics may be associated with elevated thrombotic risk.
SUMMARY
Thrombocytopenia is common in APS, and is likely associated with more severe disease. Improved understanding of thrombocytopenia in APS has the potential to improve risk stratification, reveal novel aspects of APS pathophysiology, and lead to treatments that are more individualized and holistic.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Female; Humans; Male; Pregnancy; Thrombocytopenia
PubMed: 30747734
DOI: 10.1097/BOR.0000000000000595 -
Autoimmunity Reviews Aug 2022APS (antiphospholipid syndrome) is a systematic autoimmune disease accompanied with venous or arterial thrombosis and poor pregnant manifestations, partly attributing to... (Review)
Review
APS (antiphospholipid syndrome) is a systematic autoimmune disease accompanied with venous or arterial thrombosis and poor pregnant manifestations, partly attributing to the successive elevated aPL (antiphospholipid antibodies) and provoked prothrombotic and proinflammatory molecules production. Nowadays, most researches focus on the laboratory detection and clinic features of APS, but its precise etiology remains to be deeply explored. As we all know, the dysfunction of ECs (endothelial cells), monocytes, platelets, trophoblasts and neutrophils are key contributors to APS progression. Especially, their epigenetic variations, mainly including the promoter CpGs methylation, histone PTMs (post-translational modifications) and ncRNAs (noncoding RNAs), result in genes expression or silence engaged in inflammation initiation, thrombosis formation, autoimmune activation and APOs (adverse pregnancy outcomes) in APS. Given the potential of epigenetic markers serving as diagnostic biomarkers or therapeutic targets of APS, and the encouraging advancements in epigenetic drugs are being made. In this review, we would systematically introduce the epigenetic underlying mechanisms for APS progression, comprehensively elucidate the functional mechanisms of epigenetics in boosting ECs, monocytes, platelets, trophoblasts and neutrophils. Lastly, the application of epigenetic alterations for probing novel diagnostic, specific therapeutic and prognostic strategies would be proposed.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Endothelial Cells; Epigenesis, Genetic; Female; Humans; Pregnancy; Thrombosis
PubMed: 35690246
DOI: 10.1016/j.autrev.2022.103130