Antiphospholipid syndrome (APS), also known as Hughes Syndrome, is a systemic autoimmune disease characterized by thrombosis and/or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. A patient with APS must meet at least one of two clinical criteria (vascular thrombosis or complications of pregnancy) and at least one of two laboratory criteria including the persistent presence of lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and/or anti-b2 glycoprotein I (anti-b2GPI) antibodies of IgG or IgM isotype at medium to high titres in patient's plasma. However, several other autoantibodies targeting other coagulation cascade proteins (i.e. prothrombin) or their complex with phospholipids (i.e. phosphatidylserine/prothrombin complex), or to some domains of β2GPI, have been proposed to be also relevant to APS. In fact, the value of testing for new aPL specificities in the identification of APS in thrombosis and/or pregnancy morbidity patients is currently being investigated.
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http://dx.doi.org/10.12688/f1000research.9717.1 | DOI Listing |
Lupus Sci Med
January 2025
Medicine, University of Washington, Seattle, Washington, USA.
Objectives: To study neutrophil gelatinase-associated lipocalin (NGAL) levels in peripheral blood in SLE, and to propose a mechanism by which neutrophils secrete NGAL on stimulation with immune complexes (IC).
Methods: NGAL was measured by ELISA in two independent Swedish SLE cohorts acting as exploratory and validation cohort (n=124 and n=308, respectively), disease controls (n=38) and healthy controls (n=77). NGAL levels were measured in supernatant from IC-stimulated neutrophils in the presence or absence of a toll-like receptor 8 inhibitor (TLR8i).
J Autoimmun
January 2025
Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark; Department of Cardiology, Copenhagen University Hospital, Herlev-Gentofte Hospital, Copenhagen, Denmark.
Introduction: Maternal autoimmune systemic connective tissue diseases (CTDs) and their related antibodies have been associated with adverse fetal outcomes, including complete heart block. In this study, we assessed the association between maternal CTD or vasculitis and neonatal electrocardiographic (ECG) parameters.
Methods: Our study population was drawn from the Copenhagen Baby Heart Study (CBHS), a prospective, population-based cohort study open to all neonates born in the Copenhagen area.
Eur J Case Rep Intern Med
December 2024
Department of Internal Medicine, Ospedale Regionale di Lugano EOC, Lugano, Switzerland.
Unlabelled: This article describes a case of a 26-year-old female with a history of Evan's syndrome who presented with severe exertional dyspnoea and abdominal discomfort. The patient was diagnosed with chronic Budd-Chiari syndrome, a rare vascular disorder characterized by obstruction of the hepatic vein. We discuss the risk factors, the clinical manifestations, and diagnostic methods for Budd-Chiari syndrome, as well as the possible association with an underlying incomplete systemic lupus erythematosus.
View Article and Find Full Text PDFEur J Case Rep Intern Med
December 2024
Radiology Department, Seychelles Hospital, Healthcare Agency, Victoria, Seychelles.
Unlabelled: Upper extremity deep vein thrombosis (UEDVT) is relatively rare, and much less as an initial presentation of systemic lupus erythematosus (SLE). Primary UEDVT should be considered in individuals with unilateral arm swelling where the brachial, axillary, and subclavian veins are frequently involved. SLE is a chronic autoimmune disease that predominantly affects women of childbearing age and of African descent.
View Article and Find Full Text PDFCureus
December 2024
Neurology and Neuroscience, Medical University of Varna, Varna, BGR.
Neuromyelitis optica spectrum disorder (NMOSD) includes conditions with autoimmune genesis, which are manifested by attacks of optic neuritis (ON) and transverse myelitis (TM), and also express aquaporin 4 (NMO-IgG) or myelin oligo-endocytic glycoprotein (MOGAb) antibodies. In rare cases, the disease may also have a clinical presentation with only TM, without ON or with ON, without TM. These conditions are also included in the spectrum.
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