5,972 results match your criteria Immunoglobulin G Deficiency

Collagenous Gastritis in Primary Selective IgM Deficiency: Transition to EBV+ Gastric Adenocarcinoma.

Case Reports Immunol 2021 25;2021:5574944. Epub 2021 May 25.

Division of Basic and Clinical Immunology, University of California, Irvine, California, USA.

Selective IgM deficiency (SIgMD) and isolated collagenous gastritis are two independent rare disorders. Our purpose is to report the 1 case of SIgMD and isolated collagenous gastritis and collagenous gastritis that has transitioned to EBV + gastric adenocarcinoma. Gastric biopsy tissue was analyzed by EBV-related encoded RNA in situ hybridization assay. Read More

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Genetic Variation in Two Populations From Ecuador Highlands-Extensive Copy-Number Variation, Distinctive Distribution of Functional Polymorphisms, and a Novel, Locally Common, Chimeric (CD16B/A) Gene.

Front Immunol 2021 24;12:615645. Epub 2021 May 24.

Immunogenetics & Histocompatibility Lab, Instituto de Investigación Sanitaria Puerta de Hierro-Segovia de Arana, Majadahonda, Spain.

Fcγ receptors (FcγR), cell-surface glycoproteins that bind antigen-IgG complexes, control both humoral and cellular immune responses. The locus on chromosome 1q23.3 comprises five homologous genes encoding low-affinity FcγRII and FcγRIII, and displays functionally relevant polymorphism that impacts on human health. Read More

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SARS-CoV-2 infectious virus, viral RNA in nasopharyngeal swabs, and serostatus of symptomatic COVID-19 outpatients in the United States.

medRxiv 2021 Jun 1. Epub 2021 Jun 1.

Background: SARS-CoV-2 infectious virus isolation in the upper airway of COVID-19 patients is associated with higher levels of viral RNA. However, comprehensive evaluation of the relationships between host and disease factors and infectious, replication competent virus is needed.

Methods: Symptomatic COVID-19 outpatients were enrolled from the United States. Read More

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The protective effect of Angiotensin AT2-receptor stimulation in Neuromyelitis optica spectrum disorder is independent of astrocyte-derived BDNF.

Mult Scler Relat Disord 2021 May 21;53:103033. Epub 2021 May 21.

Department of Neurobiology Research, Institute of Molecular Medicine, University of Southern Denmark, Odense, Denmark; Brain Research InterDisciplinary Guided Excellence (BRIDGE), University of Southern Denmark, Denmark. Electronic address:

Background: Neuromyelitis optica spectrum disorder (NMOSD) is an antibody-mediated autoimmune inflammatory disease of the central nervous system (CNS), resulting in primary astrocytopathy. We have previously shown that Angiotensin AT2-receptor (AT2R) stimulation with the specific agonist Compound 21 (C21) attenuated NMOSD-like pathology. Recent studies have proposed that the mechanism behind protective effects of AT2R includes induction of brain derived neurotrophic factor (BDNF). Read More

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Tolerability and Efficacy of s.c. IgG Self-Treatment in ME/CFS Patients with IgG/IgG Subclass Deficiency: A Proof-of-Concept Study.

J Clin Med 2021 May 29;10(11). Epub 2021 May 29.

Institute of Medical Immunology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Augustenburger Platz 1, 13353 Berlin, Germany.

Background: Chronic fatigue syndrome (ME/CFS) is a complex disease frequently triggered by infections. IgG substitution may have therapeutic effect both by ameliorating susceptibility to infections and due to immunomodulatory effects.

Methods: We conducted a proof of concept open trial with s. Read More

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Effectiveness of low-dose intravenous immunoglobulin therapy in minor primary antibody deficiencies: a two-year real-life experience.

Clin Exp Immunol 2021 Jun 1. Epub 2021 Jun 1.

Immunoallergology Unit, Careggi University Hospital, Florence, Italy.

Primary Antibody Deficiencies (PAD) are the most prevalent group of Primary Immunodeficiencies (PID) in adults and Immunoglobulin Replacement Therapy (IRT) is the mainstay therapy to improve clinical outcomes. IRT is, however, expensive and, in minor PAD, clear recommendations about IRT are lacking. We conducted a retrospective real-life study to assess the effectiveness of low-dose IRT in minor PAD on 143 patients fulfilling European Society for Immunodeficiencies (ESID) diagnostic criteria for IgG subclass deficiency (IgGSD) or Unclassified Antibody Deficiency (UAD). Read More

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Acquired hypofibrinogenemia in a patient with multiple myeloma.

Int J Hematol 2021 May 31. Epub 2021 May 31.

Department of Hematology, Kitano Hospital, 2-4-20, Ougimachi, Kita-ku, Osaka, 530-8480, Japan.

We report a case of acquired hypofibrinogenemia with multiple myeloma presenting λ-type IgG monoclonal protein. The patient had anemia and renal deficiency, and also developed bleeding tendency due to severe coagulopathy. Her fibrinogen level was under the detectable limits in a functional assay. Read More

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USP13 Deficiency Aggravates Cigarette-smoke-induced Alveolar Space Enlargement.

Cell Biochem Biophys 2021 May 25. Epub 2021 May 25.

Department of Physiology and Cell Biology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.

Alveolar enlargement is a pathological feature of emphysema. Long-term exposure to cigarette smoke (CS) is a high-risk factor for the development of emphysema. Abnormal protein ubiquitination has been implicated to regulate the development of human disorders, however, the role of protein ubiquitination in emphysema has not been well-studied. Read More

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Assessment of the Association of Vitamin D Level With SARS-CoV-2 Seropositivity Among Working-Age Adults.

JAMA Netw Open 2021 05 3;4(5):e2111634. Epub 2021 May 3.

Quest Diagnostics, San Juan Capistrano, California.

Importance: Low vitamin D levels have been reported to be associated with increased risk of SARS-CoV-2 infection. Independent, well-powered studies could further our understanding of this association.

Objective: To examine whether low levels of vitamin D are associated with SARS-CoV-2 seropositivity, an indicator of previous infection. Read More

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PTIP Deficiency in B Lymphocytes Reduces Subcutaneous Fat Deposition in Mice.

Biochemistry (Mosc) 2021 05;86(5):568-576

Wuxi School of Medicine, Jiangnan University, Wuxi, 214122, China.

Recent studies have predominantly focused on the role of B cells in metabolic diseases, yet the function of B cells in adipose homeostasis remains unclear. Pax transactivation domain-interacting protein (PTIP), a licensing factor for humoral immunity, is necessary for B cell development and activation. Here, using mice that lack PTIP in B cells (PTIP mice), we explored the role of B cells in adipose homeostasis under physiological conditions. Read More

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Innate Mechanisms in Selective IgA Deficiency.

Front Immunol 2021 26;12:649112. Epub 2021 Apr 26.

Cell Biology and Immunology Group, Wageningen University & Research, Wageningen, Netherlands.

Selective IgA deficiency (SIgAD), characterized by a serum IgA level below 0.07 mg/ml, while displaying normal serum levels of IgM and IgG antibodies, is the most frequently occurring primary immunodeficiency that reveals itself after the first four years after birth. These individuals with SIgAD are for the majority healthy and even when they are identified they are usually not investigated further or followed up. Read More

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Vitamin D status of children with Paediatric Inflammatory Multisystem Syndrome Temporally associated with Severe acute respiratory syndrome coronavirus 2 (PIMS-TS).

Br J Nutr 2021 May 12:1-26. Epub 2021 May 12.

Department of General Paediatrics, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom.

Coronavirus disease 2019 (COVID-19), has caused mild illness in children, until the emergence of the novel hyperinflammatory condition PIMS-TS: Paediatric Inflammatory Multisystem Syndrome Temporally associated with Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). PIMS-TS is thought to be a post- SARS-CoV-2 immune dysregulation with excessive inflammatory cytokine release. We studied 25 hydroxyvitamin D (25OHD) concentrations in children with PIMS-TS, admitted to a tertiary paediatric hospital in the United Kingdom (U. Read More

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Isolated IgG2 Deficiency is an Independent Risk Factor for Exacerbations in Bronchiectasis.

QJM 2021 May 10. Epub 2021 May 10.

Centre for Inflammation Research at the University of Edinburgh, Queen's Medical Research Institute, Edinburgh BioQuarter, Edinburgh, EH16 4TJ, Scotland.

Background: Immunoglobulin G (IgG) subclass 2 deficiency is the most frequent IgG subclass deficiency identified in patients with bronchiectasis, but its clinical significance is not known.

Aim: To analyse if bronchiectasis patients with isolated IgG2 deficiency at risk of recurrent exacerbations and/or hospitalisation? Do patients with IgG2 deficiency have worse disease progression?

Design And Methods: This is a retrospective study (2015-2020) exploring independent risk factors for recurrent exacerbations (three or more per year) and/or hospitalisation with bronchiectasis exacerbations using multivariable models using binary logistic regression. There was no patient with IgG deficiency, IgG 1, 3 or 4 deficiency, or IgA or IgM deficiency included. Read More

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Coexistence of Acquired Hemophilia and Antiphospholipid Serology in Monoclonal Gammopathy Patient.

Int Med Case Rep J 2021 28;14:261-264. Epub 2021 Apr 28.

Department of Internal Medicine, Groupe Hospitalier Sud-Ile de France, Melun, 77000, France.

Acquired hemophilia is a rare coagulopathy with hemorrhage into the skin, muscle, or soft tissues and mucous membranes and caused by inhibitor antibodies, mainly against FVIII. We report a case of acquired hemophilia presenting with diffuse cutaneous hemorrhage and hemothorax. The patient was found to have acquired an FVIII inhibitor and a high titer of anti β2 glycoprotein 1 IgG and IgM, and anticardiolipin IgM in the context of IgA kappa-type monoclonal gammopathy. Read More

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NOX1/NADPH oxidase is involved in the LPS-induced exacerbation of collagen-induced arthritis.

J Pharmacol Sci 2021 Jun 29;146(2):88-97. Epub 2021 Jan 29.

Department of Pharmacology, Kyoto Prefectural University of Medicine, Kyoto, Japan.

We investigate as yet an unidentified role of NOX1, a non-phagocytic isoform of the superoxide-generating NADPH oxidase, in immune responses using Nox1-knockout mice (Nox1-KO). The transcripts of NOX1 was expressed in lymphoid tissues, including the spleen, thymus, bone marrow, and inguinal lymphoid nodes. When antibody production after ovalbumin (OVA) immunization was examined, no significant differences were observed in serum anti-OVA IgG levels between wild-type mice (WT) and Nox1-KO. Read More

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Lipopolysaccharide-Enhanced Responses against Aryl Hydrocarbon Receptor in FcgRIIb-Deficient Macrophages, a Profound Impact of an Environmental Toxin on a Lupus-Like Mouse Model.

Int J Mol Sci 2021 Apr 18;22(8). Epub 2021 Apr 18.

Department of Microbiology, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand.

Fc gamma receptor IIb (FcgRIIb) is the only inhibitory-FcgR in the FcgR family, and FcgRIIb-deficient (FcgRIIb) mice develop a lupus-like condition with hyper-responsiveness against several stimulations. The activation of aryl hydrocarbon receptor (Ahr), a cellular environmental sensor, might aggravate activity of the lupus-like condition. As such, 1,4-chrysenequinone (1,4-CQ), an Ahr-activator, alone did not induce supernatant cytokines from macrophages, while the 24 h pre-treatment by lipopolysaccharide (LPS), a representative inflammatory activator, prior to 1,4-CQ activation (LPS/1,4-CQ) predominantly induced macrophage pro-inflammatory responses. Read More

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Immunoglobulin deficiencies in treated patients suffering from rheumatoid arthritis.

Rheumatol Int 2021 Jul 29;41(7):1273-1280. Epub 2021 Apr 29.

Ambulantes Rheumazentrum Erfurt, Tschaikowskistraße 22, 99096, Erfurt, Germany.

Immunoglobulins and antibodies to immunoglobulins (autoimmunoglobulins) have been identified to be implicated in the pathogenesis of rheumatoid arthritis (RA). Immunoglobulin deficiencies have been suggested to account for the increased risk of infections in RA patients. This study was carried out to determine the prevalence of immunoglobulin deficiencies in patients with RA and the identification of putative contributing factors. Read More

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Immunodeficiency, Centromeric Region Instability, and Facial Anomalies Syndrome (ICF) in a Boy with Variable Clinical and Immunological Presentations.

Iran J Allergy Asthma Immunol 2021 Apr 17;20(2):249-254. Epub 2021 Apr 17.

Department of Allergy and Clinical Immunology, Iran University of Medical Sciences, Tehran, Iran.

Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare primary immunodeficiency disorder characterized by recurrent infections and low immunoglobulin levels due to variable combined immunodeficiency, and centromeric region instability, and facial dysmorphism. We describe a 12-year-old boy with recurrent respiratory tract infections, facial anomalies, scoliosis, and psychomotor retardation. He had recurrent pneumonia with low serum IgG and IgM levels during infancy and preschool age. Read More

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Positive Predictive Value of Myelin Oligodendrocyte Glycoprotein Autoantibody Testing.

JAMA Neurol 2021 Jun;78(6):741-746

Department of Neurology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota.

Importance: Myelin oligodendrocyte glycoprotein-IgG1-associated disorder (MOGAD) is a distinct central nervous system-demyelinating disease. Positive results on MOG-IgG1 testing by live cell-based assays can confirm a MOGAD diagnosis, but false-positive results may occur.

Objective: To determine the positive predictive value (PPV) of MOG-IgG1 testing in a tertiary referral center. Read More

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Compensatory IgM to the Rescue: Patients with Selective IgA Deficiency Have Increased Natural IgM Antibodies to MAA-LDL and No Changes in Oral Microbiota.

Immunohorizons 2021 Apr 23;5(4):170-181. Epub 2021 Apr 23.

Medical Microbiology and Immunology, Research Unit of Biomedicine, University of Oulu, Oulu, Finland.

IgA is the most abundant Ab in the human body. However, most patients with selective IgA deficiency (SIgAD) are asymptomatic. IgM, and to lesser extent IgG Abs, are generally presumed to compensate for the lack of IgA in SIgAD by multiplying and adopting functions of IgA. Read More

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The clinical relevance of IgM and IgA anti-pneumococcal polysaccharide ELISA assays in patients with suspected antibody deficiency.

Clin Exp Immunol 2021 Apr 20. Epub 2021 Apr 20.

Department of Tranzo, Tilburg University, Tilburg, the Netherlands.

Unlike immunoglobulin (Ig)G pneumococcal polysaccharide (PnPS)-antibodies, PnPS IgA and IgM-antibodies are not routinely determined for the assessment of immunocompetence. It is not yet known whether an isolated inability to mount a normal IgM or IgA-PnPS response should be considered a relevant primary antibody deficiency (PAD). We studied the clinical relevance of anti-PnPS IgM and IgA-assays in patients with suspected primary immunodeficiency in a large teaching hospital in 's-Hertogenbosch, the Netherlands. Read More

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Individuals with isolated congenital GH deficiency due to a GHRH receptor gene mutation appear to cope better with SARS-CoV-2 infection than controls.

Endocrine 2021 05 16;72(2):349-355. Epub 2021 Apr 16.

Division of Endocrinology, Health Sciences Graduate Program, Aracaju, Brazil.

Purpose: Several interactions exist between the GH/IGF axis and the immune system, including effects on innate immunity and humoral and cellular response. Acquired GH deficiency (GHD) has been recently proposed as a risk factor for severity of COVID-19 infections. However, acquired GHD is often associated to other factors, including pituitary tumors, surgery, radiotherapy, and additional pituitary hormones deficits and their replacements, which, together, may hinder an accurate analysis of the relationship between GHD and COVID-19. Read More

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Clinical heterogeneity among pediatric patients with autoimmune type 1 diabetes stratified by immunoglobulin deficiency.

Pediatr Diabetes 2021 Apr 10. Epub 2021 Apr 10.

Department of Pediatrics, Oncology, and Hematology, Medical University of Łódź, Łódź, Poland.

Background: Type 1 diabetes (T1D) may coexist with primary immunodeficiencies, indicating a shared genetic background.

Objective: To evaluate the prevalence and clinical characteristics of immunoglobulin deficiency (IgD) among children with T1D.

Methods: Serum samples and medical history questionnaires were obtained during routine visits from T1D patients aged 4-18 years. Read More

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A human monoclonal antibody against the distal carboxyl terminus of ADAMTS-13 modulates its susceptibility to an inhibitor in thrombotic thrombocytopenic purpura.

J Thromb Haemost 2021 Apr 9. Epub 2021 Apr 9.

Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, Kansas, USA.

Background: Immune thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal thrombotic microangiopathy, resulting from a severe deficiency of plasma ADAMTS-13 (A Disintegrin And Metalloproteinase with ThromboSpondin type 1 motif, member 13) activity. IgG-type autoantibodies are primarily responsible for the inhibition of plasma ADAMTS-13 activity. However, the mechanism underlying autoantibody-mediated inhibition is not fully understood. Read More

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Recovery from acute SARS-CoV-2 infection and development of anamnestic immune responses in T cell-depleted rhesus macaques.

bioRxiv 2021 Apr 4. Epub 2021 Apr 4.

Severe COVID-19 has been associated with T cell lymphopenia 1,2, but no causal effect of T cell deficiency on disease severity has been established. To investigate the specific role of T cells in recovery from SARS-CoV-2 infections we studied rhesus macaques that were depleted of either CD4+, CD8+ or both T cell subsets prior to infection. Peak virus loads were similar in all groups, but the resolution of virus in the T cell-depleted animals was slightly delayed compared to controls. Read More

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[Acupuncture combined with powder for children with cerebral palsy and its effect on serum immune indexes and nerve growth related protein].

Zhongguo Zhen Jiu 2021 Mar;41(3):288-92

Rehabilitation Center, Children's Hospital of Zhengzhou University/Henan Children's Hospital/Zhengzhou Children's Hospital, Zhengzhou 450002, China.

Objective: To observe the efficacy of acupuncture combined with powder for children with cerebral palsy (liver- stagnation, spleen-kidney deficiency syndrome) and its effect on serum immune indexes and nerve growth related protein.

Methods: A total of 180 children with cerebral palsy were randomly divided into a combined group (60 cases, 2 cases dropped off), an acupuncture group (60 cases, 4 cases dropped off) and a Chinese medication group (60 cases, 5 cases dropped off). On the basis of conventional treatment, the children in the combined group were treated with acupuncture [Baihui (GV 20), Sishencong (EX-HN 1), Shenting (GV 24), Benshen (GB 13), 30 min each time, twice a day] and powder; the children in the acupuncture group were treated with acupuncture, and the children in the Chinese medication group were treated with powder, the treatment was same with the combined group. Read More

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Interstitial Lung Disease in Common Variable Immunodeficiency.

Front Immunol 2021 11;12:605945. Epub 2021 Mar 11.

Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, United States.

Interstitial lung disease (ILD) is a common complication in patients with common variable immunodeficiency (CVID) and often associated with other features, such as bronchiectasis and autoimmunity. As the ILD term encompasses different acute and chronic pulmonary conditions, the diagnosis is commonly made based on imaging features; histopathology is less frequently available. From a cohort of 637 patients with CVID followed at our center over 4 decades, we reviewed the data for 46 subjects (30 females, 16 males) who had lung biopsies with proven ILD. Read More

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Autoantibody of NRIP, a novel AChR-interacting protein, plays a detrimental role in myasthenia gravis.

J Cachexia Sarcopenia Muscle 2021 Jun 26;12(3):665-676. Epub 2021 Mar 26.

Graduate Institute of Microbiology, College of Medicine, National Taiwan University, Taipei, Taiwan.

Background: Nuclear receptor interaction protein (NRIP) co-localizes with acetylcholine receptor (AChR) at the neuromuscular junction (NMJ), and NRIP deficiency causes aberrant NMJ architecture. However, the normal physiological and pathophysiological roles of NRIP in NMJ are still unclear.

Methods: We investigated the co-localization and interaction of NRIP with AChR-associated proteins using immunofluorescence and immunoprecipitation assay, respectively. Read More

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ICOS ligand and IL-10 synergize to promote host-microbiota mutualism.

Proc Natl Acad Sci U S A 2021 Mar;118(13)

Department of Pathology, University of Alabama at Birmingham, Birmingham, AL 35294;

Genome-wide association studies have identified , which encodes the inducible costimulator igand (ICOSLG or ICOSL) as a susceptibility locus for inflammatory bowel disease. ICOSL has been implicated in the enhancement of pattern recognition receptor signaling in dendritic cells, induction of IL-10 production by CD4 T cells, and the generation of high-affinity antibodies to specific antigens-all of which can potentially explain its involvement in gastrointestinal inflammation. Here, we show that murine ICOSL deficiency results in significant enrichment of IL-10-producing CD4 T cells particularly in the proximal large intestine. Read More

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Beta-2-Glycoprotein-I Deficiency Could Precipitate an Antiphospholipid Syndrome-like Prothrombotic Situation in Patients With Coronavirus Disease 2019.

ACR Open Rheumatol 2021 Apr 19;3(4):267-276. Epub 2021 Mar 19.

Hospital 12 de Octubre, Healthcare Research Institute and Biomedical Research Centre Network for Epidemiology and Public Health, Madrid, Spain.

Objective: Patients with coronavirus disease 2019 (COVID-19) present coagulation abnormalities and thromboembolic events that resemble antiphospholipid syndrome (APS). This work has aimed to study the prevalence of APS-related antigens, antibodies, and immune complexes in patients with COVID-19 and their association with clinical events.

Methods: A prospective study was conducted on 474 adults with severe acute respiratory syndrome coronavirus 2 infection hospitalized in two Spanish university hospitals. Read More

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