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Semin Thromb Hemost 2021 Jun 15. Epub 2021 Jun 15.

Section of Clinical Biochemistry, University of Verona, Verona, Italy.

The term "lupus anticoagulant (LA)" identifies a form of antiphospholipid antibodies (aPLs) causing prolongation of clotting tests in a phospholipid concentration-dependent manner. LA is one of the laboratory criteria identified in patients with antiphospholipid (antibody) syndrome (APS). The presence of LA in patients with APS represents a significant risk factor for both thrombosis and pregnancy morbidity. Read More

Semin Thromb Hemost 2021 Jun 15. Epub 2021 Jun 15.

Section of Clinical Biochemistry, University of Verona, Verona, Italy.

Antiphospholipid antibodies (aPL) comprise a panel of autoantibodies that reflect a potential prothrombotic risk in several autoimmune conditions, most notably antiphospholipid (antibody) syndrome (APS). aPL can be divided into those that form part of the laboratory criteria for APS, namely, lupus anticoagulant (LA), as well as anticardiolipin antibodies (aCL) and anti-β2-glycoprotein I antibodies (aβ2GPI) of the immunoglobulin G and M classes, and those that form a group considered as "noncriteria antibodies." The noncriteria antibodies include, for example, antiphosphatidylserine antibodies (aPS), antiprothrombin antibodies (aPT), and antiphosphatidylserine/prothrombin complex antibodies (aPS/PT). Read More

Curr Neurol Neurosci Rep 2021 Jun 14;21(8):41. Epub 2021 Jun 14.

Neurology, Department of Neurosciences and Mental Health, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal.

Purpose Of Review: In recent years, the spectrum of neurological manifestations of antiphospholipid syndrome (APS) has been growing. We provide a critical review of the literature with special emphasis on presentation, proposed mechanisms of disease, and treatment of neurological involvement in APS.

Recent Findings: Although stroke is the most common cause of neurological manifestations in patients with APS, other neurological disorders have been increasingly associated with the disease, including cognitive dysfunction, headache, and epilepsy. Read More

Eur J Case Rep Intern Med 2021 24;8(5):002610. Epub 2021 May 24.

Department Gastroenterology and Hepatology, Baylor College of Medicine, Houston, Texas, USA.

Antiphospholipid syndrome (APS) is a multisystemic autoimmune disease which presents with thromboembolic disease, pregnancy complications and the presence of antiphospholipid antibodies. There are some reports of arterial dissections in different sites of the body associated with APS. We describe two patients with APS who developed ischaemic stroke as a result of vertebral artery dissection in the absence of acquired and genetic risk factors for arterial dissection. Read More

Ann Palliat Med 2021 Jun 8. Epub 2021 Jun 8.

Department of Rheumatology, the First Hospital of Hebei Medical University, Shijiazhuang, China.

Antiphospholipid syndrome (APS) is an autoimmune systemic disorder characterized by arterial, venous, or small vessel thrombosis, and/or recurrent early pregnancy loss, fetal loss, or pregnancy morbidity. APS is induced by persistent positive antiphospholipid antibodies (aPL), the main being lupus anticoagulant (LA), and/or anticardiolipin (aCL) antibodies, and/or anti-beta 2 glycoprotein 1 (β2-GP1) antibodies. Some studies have shown that the incidence of APS is about 5 new cases per 100,000 persons per year, and the prevalence is around 40-50 cases per 100,000 persons. Read More

Joint Bone Spine 2021 Jun 8:105236. Epub 2021 Jun 8.

Department of Medicine, Universitat de Barcelona, Barcelona, Spain; Department of Autoimmune Diseases, ICMiD, Hospital Clinic, Barcelona, Spain. Electronic address:

Objective: To analyze whether immune-mediated diseases (IMDs) occurs in sarcoidosis more commonly than expected in the general population, and how concomitant IMDs influence the clinical presentation of the disease.

Methods: We searched for coexisting IMDs in patients included in the SARCOGEAS-cohort, a multicenter nationwide database of consecutive patients diagnosed according to the ATS/ESC/WASOG criteria. Comparisons were made considering the presence or absence of IMD clustering, and odds ratios (OR) and their 95% confidence intervals (CI) were calculated as the ratio of observed cases of every IMD in the sarcoidosis cohort to the observed cases in the general population. Read More

Rheumatology (Oxford) 2021 Jun 11. Epub 2021 Jun 11.

Universidad Autonoma de Barcelona, Vall d´Hebron University Hospital, Barcelona, Spain.

Objectives: To investigate the impact of thrombocytopenia on survival in patients with antiphospholipid syndrome (APS).

Methods: Thrombocytopenia and other predictors of outcome were retrospectively evaluated in an antiphospholipid-antibody (aPL)-positive and APS cohort with 38-year follow-up (1980-2018). Thrombocytopenia was defined as < 150 × 109 platelets/l. Read More

Curr Opin Pulm Med 2021 Jun 9. Epub 2021 Jun 9.

Department of Medicine, University of Alberta, Clinical Sciences Building, Edmonton, Alberta Department of Medicine, Division of Hematology, Ottawa Research Hospital Institute Department of Medicine, University of Ottawa, The Ottawa Research Hospital Institute, The Ottawa Hospital General Campus, Ottawa, Ontario, Canada.

Purpose Of Review: The pivotal phase III trials demonstrating efficacy and safety of direct oral anticoagulants (DOACs) in the treatment of venous thromboembolism (VTE) or nonvalvular atrial fibrillation (NVAF) excluded patients with important and common comorbidities, including obesity, advanced chronic kidney disease, cirrhosis, cancer and antiphospholipid antibody syndrome. Despite the lack of large prospective randomized control trials in these patient populations, the use of DOACs has led to a wealth of efficacy and safety data within these groups.

Recent Findings: Retrospective studies, meta-analyses, national databases and pharmacokinetic data have shed light on the efficacy and safety of DOACs in these patient populations. Read More

Arthritis Rheumatol 2021 Jun 11. Epub 2021 Jun 11.

Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery; Weill Cornell Medicine, New York, NY, USA.

A 50-year-old male with antiphospholipid syndrome (APS) presents to your office for the first time with a three-month history of worsening dyspnea, dry cough, and early morning blood-tinged sputum. His APS diagnosis was based on an unprovoked left lower extremity deep vein thrombosis, four years prior to the presentation, with persistent (multiple occasions) triple antiphospholipid antibody (aPL)-positivity, defined as lupus anticoagulant (LA) test positive performed while off anticoagulation, high titer [≥80 U] IgG anticardiolipin antibodies (aCL), and high titer IgG anti-β -glycoprotein-I (aβ GPI). He has no other past medical history; and he is on warfarin with a target international normalized ratio (INR) of 2. Read More

Eur Rev Med Pharmacol Sci 2021 May;25(10):3645-3646

Serviço de Rheumatology Division, University of São Paulo, São Paulo-SP, Brazil.

Rinsho Ketsueki 2021 ;62(5):445-455

Department of Hematology and Clinical Laboratory, Iwate Prefectural Chubu Hospital.

Antiphospholipid syndrome (APS) is an acquired thrombophilia associated with autoimmunity and is a syndrome that should always be considered when examining patients with thrombosis and pregnancy complications. As per the Sydney criteria, a diagnosis can be established with at least one clinical finding, such as arteriovenous thrombosis and the presence of at least one of the antiphospholipid antibodies (aPL), such as anticardiolipin antibodies (aCL). Moreover, phosphatidylserine-dependent anti-prothrombin antibody and anti-b2GPI-domain 1 antibody are correlated with APS manifestations and enable APS diagnosis. Read More

Clin Exp Immunol 2021 Jun 9. Epub 2021 Jun 9.

Dipartimento di Medicina Sperimentale, Sapienza University, Rome, Italy.

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder defined by the simultaneous presence of vascular clinical events, pregnancy morbidity and antiphospholipid antibodies (aPL). In clinical practice it is possible to find patients with APS who are persistently negative for the routine aPL tests ("seronegative APS", SN-APS). Recently, the identification of aPL IgA and/or anti-β2-GPI IgA was shown to represent a further test in SN-APS patients. Read More

Semin Thromb Hemost 2021 Jun 8. Epub 2021 Jun 8.

Grupo Reproducción, Departamento Microbiología y Parasitología, Facultad de Medicina, Universidad de Antioquia UdeA, Medellín, Colombia.

Eur J Intern Med 2021 Jun 4. Epub 2021 Jun 4.

Hematology and Hemotherapy Center, University of Campinas, Campinas, SP, Brazil; School of Medical Sciences, Department of Pathology, University of Campinas, Campinas, SP, Brazil. Electronic address:

Clin Investig Arterioscler 2021 Jun 4. Epub 2021 Jun 4.

Unidad de Riesgo Vascular, Medicina Interna, Hospital Universitario Virgen de las Nieves, Granada, España.

Renal infarction is a rare disease whose incidence is less than 1%. The symptoms can be abdominal or flank pain, nausea, vomiting, fever or hypertension. The diagnosis is complex, and it is based on symptoms, blood analysis with an elevated level of lactate dehydrogenase and computed tomography angiography. Read More

J Autoimmun 2021 Jun 5;122:102675. Epub 2021 Jun 5.

Department of Infectious Disease, Immunology and Sexual Health, St George Hospital and Department of Medicine, St George and Sutherland Clinical School, University of New South Wales, Sydney, NSW, Australia; Department of Rheumatology, St George Hospital, University of New South Wales, Sydney, NSW, Australia. Electronic address:

Β-Glycoprotein I (βGPI) is an important anti-thrombotic protein and is the major auto-antigen in the antiphospholipid syndrome (APS). The clinical relevance of nitrosative stress in post translational modification of βGPI was examined.The effects of nitrated (n)βGPI on its anti-thrombotic properties and its plasma levels in primary and secondary APS were determined with appropriate clinical control groups. Read More

SAGE Open Med Case Rep 2021 20;9:2050313X211015895. Epub 2021 May 20.

Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Rheumatoid vasculitis is a rare extra-articular complication of rheumatoid arthritis. The most common manifestation is cutaneous; however, it can manifest in various organ systems and is associated with a high degree of morbidity and mortality. Diagnosis is challenging, and there are no validated diagnostic or classification criteria. Read More

Parkinsonism Relat Disord 2021 May 29. Epub 2021 May 29.

Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, UK; Department of Neurology, University Hospital, Heidelberg, Germany. Electronic address:

With the advances in neuroimmunology especially due to the discovery of new neuronal antibodies, the recognition of treatable antibody-related movement disorders has recently received much attention. In contrast, the identification and characterisation of movement disorders associated with systemic autoimmune diseases remains a substantially unexplored area. Beyond the classic few associations such as chorea and antiphospholipid syndrome, or ataxia and coeliac disease, movement disorders have been reported in association with several systemic autoimmune diseases, however a clear image of clinical phenotypes, investigations, and treatment outcomes in these conditions has never been drawn. Read More

Cytokine Growth Factor Rev 2021 May 28. Epub 2021 May 28.

Department of Otorhinolaryngology-Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, 3610 Hamilton Walk, 201E JP, Philadelphia, PA, 19104, USA. Electronic address:

Antiphospholipid antibodies (aPLs), present in 1-5 % of healthy individuals, are associated with the risk of antiphospholipid syndrome (APS), which is the most common form of acquired thrombophilia. APLs may appear following infections or vaccinations and have been reported in patients with COronaVIrus Disease-2019 (COVID-19). However, their association with COVID-19 vaccination is unclear. Read More

J Hum Reprod Sci 2021 Jan-Mar;14(1):97-100. Epub 2021 Mar 30.

Department of Microbiology and Parasitology, School of Medicine, Reproduction Group, University of Antioquia, Medellín, Colombia.

This is a case report of women with pregnancy morbidity (PM), some of them associated with antiphospholipid syndrome (APS), in which the glycan patterns of immunoglobulin G (IgG) were investigated based on the theory of alteration of glycosylation in autoimmunity. We used lectin blot to determine changes in terminal glycosylation of polyclonal IgG from women with antiphospholipid (aPL) antibodies and PM plus vascular thrombosis (PM/VT) and seronegative-obstetric APS (SN-OAPS). In addition, we analyzed IgG from women with PM without aPL (PM/aPL-) and healthy women, as controls. Read More

Lupus 2021 Jun 4:9612033211021488. Epub 2021 Jun 4.

Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

Most of the knowledge in pediatric antiphospholipid syndrome (APS) is derived from studies performed on the adult population. As in adults, antiphospholipid antibodies (aPL) can contribute to thrombosis, especially cerebrovascular thrombosis, in neonates and children. Since aPL have the potential to cross the placental barrier, and since the pediatric population is prone to infections, re-testing for their positivity is essential to specify their role in cerebrovascular thrombosis. Read More

Am J Clin Pathol 2021 Jun 2. Epub 2021 Jun 2.

Department of Pathology and Laboratory Medicine, University of California, San Francisco, San Francisco, CA, USA.

Objectives: Lupus anticoagulant (LA) detection requires (1) prolongation of a phospholipid (PL)-dependent clot-based screening assay, (2) noncorrection upon adding normal pooled plasma, and (3) a confirmatory PL dependency test. Paired LA assays run screening and confirmatory tests simultaneously, with their test ratio (TR) or differences used to evaluate test results. We evaluated patients whose paired testing demonstrated PL dependence suggestive of LA, yet the low PL screen was not prolonged. Read More

Diagnostics (Basel) 2021 May 26;11(6). Epub 2021 May 26.

Department of Neurology, King Edward Medical University, Lahore 54000, Pakistan.

Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke that accounts for 0.5-1.0% of all strokes. Read More

J Clin Med 2021 May 13;10(10). Epub 2021 May 13.

Department of Immunology, Hospital Universitario 12 de Octubre, 28041 Madrid, Spain.

Recurrent pregnancy loss (RPL) affects up to 6% of couples. Although chromosomal aberrations of the embryos are considered the leading cause, 50% of cases remain unexplained. Antiphospholipid Syndrome is a known cause in a few cases. Read More

Arthritis Care Res (Hoboken) 2021 May 31. Epub 2021 May 31.

Clinical Immunology, Angioedema and Allergy Unit, The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Tel Aviv, Israel.

Background: The 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 ethnicity and APS in Israel. Read More

Case Rep Rheumatol 2021 4;2021:9942668. Epub 2021 May 4.

Rocky Vista University, Parker, CO, USA.

Antiphospholipid syndrome is a rare complication of postural orthostatic tachycardia syndrome. Clinically, the presentation has overlapping symptoms of both diseases, with lightheadedness or syncope when moving from a supine to a standing position as well as blood clots, headache, or pregnancy complications in women. This case presentation involves a 39-year-old patient identified as female who has been diagnosed with POTS and elevated anticardiolipin antibodies. Read More

Lupus 2021 May 30:9612033211021161. Epub 2021 May 30.

Faculty of Medicine, University of Granada, Granada, Spain.

Immune thrombocytopenia, also known as immune thrombocytopenic purpura (ITP), has been reported as an important complication related to COVID-19.We present a 49-year-old male patient with systemic lupus erythematosus with lupus nephritis, antiphospholipid syndrome and history of ITP who developed an ITP flare in the context of COVID-19. He had no bleeding manifestations and had a good response to prednisone treatment. Read More

J Matern Fetal Neonatal Med 2021 May 27:1-8. Epub 2021 May 27.

The First I.M. Sechenov Moscow State Medical University (Sechenov University), Moscow, Russia.

Background: The current recommended therapy of obstetric antiphospholipid syndrome (APS) is a long-term anticoagulant therapy that affects the final event, namely, when the thrombosis has already occurred. Unfortunately, this schedule is not always effective and fails despite the correct risk stratification and an adequate adjusted dose.

Materials And Methods: From 2013 to 2020 we observed 217 women with antiphospholipid antibodies and obstetric morbidities who were treated with conventional treatment protocol (aspirin low doses ± LMWH). Read More

Front Immunol 2021 4;12:604222. Epub 2021 May 4.

Department of Gynecology, Shanghai First Maternity and Infant Hospital, School of Medicine, Tongji University, Shanghai, China.

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disease that can lead to thrombosis and/or pregnancy complications. Exosomes, membrane-encapsulated vesicles that are released into the extracellular environment by many types of cells, can carry signals to recipient cells to affect angiogenesis, apoptosis, and inflammation. There is increasing evidence suggesting that exosomes play critical roles in pregnancy. Read More

Lupus 2021 Jul 27;30(8):1314-1326. Epub 2021 May 27.

Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, USA.

The objectives of the 16th International Congress on Antiphospholipid Antibodies (aPL) Task Force on Clinical Manifestations of Antiphospholipid Syndrome (APS) were to critically analyze: a) the definition of "APS"; b) the current knowledge on non-traditional manifestations associated with aPL; and c) the risk stratification strategies in aPL-positive patients. The quality of evidence was assessed by the GRADE system. The task force concluded that: a) APS does not have a uniform definition given the heterogeneity of the clinical presentations and different aPL profiles; b) current literature supports the role for aPL testing in cases of thrombocytopenia and recurrent cardiac events but are limited by vast heterogeneity, providing an overall low-to-very low level of evidence; and c) risk stratification strategies in aPL-positive patients, such as aPL-Score and Global APS Score, can be useful in clinical practice. Read More