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Reumatismo Mar 2023Antiphospholipid Syndrome (APS) is an autoimmune disease which was defined in the early 1980s. The principal features include thromboembolic events and/or pregnancy...
Antiphospholipid Syndrome (APS) is an autoimmune disease which was defined in the early 1980s. The principal features include thromboembolic events and/or pregnancy losses in association with antiphospholipid antibodies (aPL). As an historical note, the full-blown picture of the syndrome resembles the illness suffered by Anne Stuart, Queen of England in the XVIII century, whose repeated miscarriages caused the end of the royal Stuart line and the Hanoverian succession. The identification of aPL started in the early XX century and was linked to the introduction of the serological test for the diagnosis of syphilis. This involves a reaction between an antibody (reagin) and a phospholipid antigen derived from bovine heart (cardiolipin). Later on, it was observed that not all subjects with a positive test had syphilis, and that the so called "false positive reaction" was often reported in patients with systemic lupus erythematosus. Different tests for the identification of aPL were subsequently developed: first lupus anticoagulant (1971) and then immunoassays for anticardiolipin (1983) and anti-beta2 glycoprotein I (1990) antibodies. In the same period the association between the presence of circulating aPL and thrombotic and obstetric events was established, both in patients with autoimmune diseases and in otherwise healthy subjects, leading to the identification of APS as a distinct autoimmune disease. This has allowed better diagnosis and more targeted treatment for many patients.
Topics: Pregnancy; Female; Humans; Animals; Cattle; Antiphospholipid Syndrome; Syphilis; Antibodies, Antiphospholipid; Lupus Coagulation Inhibitor; Lupus Erythematosus, Systemic; Autoimmune Diseases
PubMed: 36942979
DOI: 10.4081/reumatismo.2022.1556 -
Current Rheumatology Reports May 2021APS ACTION is an international research network created to design and conduct large-scale, multicenter research in persistently antiphospholipid antibody (aPL)-positive... (Review)
Review
PURPOSE OF REVIEW
APS ACTION is an international research network created to design and conduct large-scale, multicenter research in persistently antiphospholipid antibody (aPL)-positive patients. Given the expanding research activities of the network in the last decade since its creation, the purpose of this article is to review the scientific contributions of APS ACTION as well as future directions.
RECENT FINDINGS
APS ACTION has achieved increased international collaboration with internal and external investigators for outcome, interventional, and mechanistic antiphospholipid syndrome (APS) studies. This has been linked to substantial progress in Core laboratory work, which has demonstrated that laboratories can achieve good agreement in performance of aPL assays by use of the same reagents, analyzer type, and protocols. APS ACTION will continue to identify gaps in the existing aPL/APS literature, design mechanistic studies to elucidate underlying mechanisms, and conduct prospective, large-scale clinical studies, all for the ultimate goal of early diagnosis and improved management of aPL-positive patients.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Biomedical Research; Clinical Trials as Topic; Humans; Internationality; Multicenter Studies as Topic
PubMed: 33932165
DOI: 10.1007/s11926-021-01008-8 -
Pediatric Rheumatology Online Journal Feb 2022Describe the frequency of thrombotic and non-thrombotic clinical manifestations, laboratory, treatment and prognosis in patients with pediatric primary antiphospholipid... (Review)
Review
OBJECTIVE
Describe the frequency of thrombotic and non-thrombotic clinical manifestations, laboratory, treatment and prognosis in patients with pediatric primary antiphospholipid syndrome.
MATERIAL AND METHODS
A retrospective study was carried out in patients with a diagnosis of primary antiphospholipid antibody syndrome, under 16 years of age, under follow-up by the pediatric rheumatology service of the General Hospital, National Medical Center, La Raza, from January 2013 to December 2020. The antiphospholipid syndrome was defined when it met the laboratory criteria of the Sidney criteria and the presence of thrombosis or non-criteria manifestations of the disease (hematological, neurological, cutaneous, renal, cardiac or pulmonary). Demographic, clinical, laboratory, treatment, and prognosis data were collected.
RESULTS
We report 32 patients, 21 female (65%) and 11 male (35%), mean age 11.75 years, evolution time 16 weeks. Thrombosis 9 patients (28%), 1 arterial and 8 venous. Non-thrombotic manifestations; Hematologic: thrombocytopenia 22 patients (69%), autoimmune hemolytic anemia 13 (40%), Fisher-Evans syndrome 6 (19%), lupus anticoagulant with hypoprothrombinemia syndrome 2 (6%). Dermatological: livedo reticularis 20 (62%), skin ulcers 2 (6%), Raynaud's phenomenon 8 (25%). Neurological: epilepsy 1 (3%), migraine 3 (9%), chorea 1 (3%) and cognitive impairment 3 (9%). Renal in 4 (13%). Laboratory: prolonged aPTT 30 (93%), lupus anticoagulant 32 (100%), positive IgG anticardiolipin 20 (62%), positive IgM anticardiolipin 19 (60%). AntiB2GPI was performed in only 3 patients, being positive in all.
TREATMENT
anticoagulation in patients with thrombosis, antiplatelet in 23 (72%), steroid 30 (94%), immunosuppressant 30 (94%) and rituximab 4 (12.5%). No deaths were reported.
CONCLUSIONS
The clinical characteristics of patients with pediatric primary antiphospholipid syndrome differ from those presented in adults, since non-thrombotic manifestations are more frequent in children, for which classification criteria that include these manifestations are necessary for a better characterization of the disease in pediatric population.
Topics: Adolescent; Antiphospholipid Syndrome; Child; Child, Preschool; Female; Humans; Infant; Male; Retrospective Studies
PubMed: 35164787
DOI: 10.1186/s12969-022-00673-y -
Internal Medicine (Tokyo, Japan) Oct 1995
Topics: Antiphospholipid Syndrome; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic
PubMed: 8563092
DOI: 10.2169/internalmedicine.34.938 -
Seminars in Thrombosis and Hemostasis Jul 2018Persistent serum positivity for antiphospholipid antibodies (aPL) is required to diagnose antiphospholipid syndrome (APS), an autoimmune disease characterized by... (Review)
Review
Persistent serum positivity for antiphospholipid antibodies (aPL) is required to diagnose antiphospholipid syndrome (APS), an autoimmune disease characterized by recurrent vascular thrombosis and/or pregnancy morbidity. The current therapeutic management of patients with thrombotic APS aims at preventing recurrences and long-term complications by attenuating the procoagulant state. There is overall consensus to reserve moderate-intensity anticoagulation to aPL-positive patients with a previous venous thrombosis; the therapeutic options for those with a history of arterial event comprise antiplatelet agents and high-intensity anticoagulation. Unfortunately, thrombotic occurrences might occur despite adequate anticoagulation, carrying a significant burden of morbidity and mortality. The management of refractory thrombotic APS and catastrophic APS is still not clear, warranting the issue of recommendations. Vitamin-K antagonists are limited by significant side effects, and a careful weighting of risks and benefits should be performed to reserve the optimal treatment to each patient. To overcome these limitations, novel oral anticoagulants have been introduced in the market, but their efficacy in thrombotic APS has still to be unraveled. The poor safety profile and the scarce efficacy of drugs acting on the coagulation cascade explain why novel therapeutic approaches are currently under investigation, to identify pharmacological tools specifically counteracting aPL-mediated prothrombotic effects.
Topics: Anticoagulants; Antiphospholipid Syndrome; Female; Humans; Pregnancy; Risk; Thrombolytic Therapy; Thrombosis
PubMed: 28278524
DOI: 10.1055/s-0036-1597282 -
The Israel Medical Association Journal... May 2012
Topics: Antiphospholipid Syndrome; Female; Humans; Male
PubMed: 22799064
DOI: No ID Found -
Internal Medicine (Tokyo, Japan) Jun 2000
Topics: Antiphospholipid Syndrome; Heart Valve Diseases; Humans
PubMed: 10852159
DOI: 10.2169/internalmedicine.39.446 -
Clinical & Developmental Immunology 2013Antiphospholipid syndrome (APS) is an acquired thrombophilia with clinical manifestations associated with the presence of antiphospholipid antibodies (aPL) in patient... (Review)
Review
Antiphospholipid syndrome (APS) is an acquired thrombophilia with clinical manifestations associated with the presence of antiphospholipid antibodies (aPL) in patient plasma. Obstetrical APS is a complex entity that may affect both mother and fetus throughout the entire pregnancy with high morbidity. Clinical complications are as various as recurrent fetal losses, stillbirth, intrauterine growth restriction (IUGR), and preeclampsia. Pathogenesis of aPL targets trophoblastic cells directly, mainly via proapoptotic, proinflammatory mechanisms, and uncontrolled immunomodulatory responses. Actual first-line treatment is limited to low-dose aspirin (LDA) and low-molecular weight heparin (LMWH) and still failed in 30% of the cases. APS pregnancies should be a major field in obstetrical research, and new therapeutics are still in progress.
Topics: Antiphospholipid Syndrome; Female; Humans; Male; Pregnancy
PubMed: 23983765
DOI: 10.1155/2013/159124 -
Annals of Medicine Dec 2021Antiphospholipid syndrome (APS) is an autoimmune disease mainly characterised by vascular thrombosis and pregnancy morbidity. APS has broad spectrum of clinical... (Review)
Review
Antiphospholipid syndrome (APS) is an autoimmune disease mainly characterised by vascular thrombosis and pregnancy morbidity. APS has broad spectrum of clinical manifestations. The digestive system involvement of antiphospholipid syndrome is a critical but under-recognised condition. Digestive system involvement may be the result of direct (autoimmune-mediated) or indirect (thrombotic) mechanisms. Liver is the most commonly involved organ, followed by intestines, oesophagus, stomach, pancreas and spleen. This review describes possible digestive system manifestations in APS patients, and illustrates the epidemiology and possible pathophysiology of APS. The role of different treatment strategies in the management of digestive system manifestations of APS were also discussed.Key messagesAntiphospholipid syndrome is a multi-organ, multi-system disease and its clinical manifestation spectrum is gradually expanding. Since the first diagnosis of APS, the clinical manifestations of digestive system have been reported successively. This narrative review describes the major digestive system manifestations of APS and illustrates the epidemiology, pathophysiology and the role of therapeutic strategies of these patients.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Autoimmune Diseases; Digestive System; Female; Humans; Pregnancy; Thrombosis
PubMed: 34409894
DOI: 10.1080/07853890.2021.1962964 -
Hematology. American Society of... Dec 2019Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease, the obstetric features of which include recurrent early miscarriage, fetal death at or beyond 10... (Review)
Review
Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease, the obstetric features of which include recurrent early miscarriage, fetal death at or beyond 10 weeks of gestation, and early delivery for severe preeclampsia or placental insufficiency. Controversies regarding the specificity of these obstetric clinical features, as well as the laboratory diagnostic criteria, are the subject of current debate and reanalysis. Clinical and laboratory features can be used to stratify women with APS in terms of risk of adverse second and third trimester pregnancy outcomes. Numerous "treatments" have been used in high-risk and refractory patients, but rigorously designed clinical trials are needed. APS is a rare disease that requires innovative investigative approaches to provide credible results.
Topics: Adult; Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Female; Humans; Placental Insufficiency; Pre-Eclampsia; Pregnancy
PubMed: 31808896
DOI: 10.1182/hematology.2019000043