13,593 results match your criteria Antiphospholipid Antibody Syndrome and Pregnancy


Antiphospholipid score is a novel risk factor for idiopathic osteonecrosis of the femoral head in patients with systemic lupus erythematosus.

Rheumatology (Oxford) 2018 Dec 6. Epub 2018 Dec 6.

Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

Objectives: Idiopathic osteonecrosis of the femoral head (ION) is a common complication of SLE associated with CS therapy. Although the pathogenesis of ION involves local bone ischaemia favoured by thrombophilia, the involvement of aPL in lupus ION remains to be elucidated. We have previously reported the aPL score (aPL-S) as a quantitative marker of aPL and the development of thrombotic events in autoimmune diseases. Read More

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December 2018

Efficacy of dual antiplatelet therapy for preventing recurrence of arterial thrombosis in patients with antiphospholipid syndrome.

Rheumatology (Oxford) 2018 Nov 30. Epub 2018 Nov 30.

Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

Objective: Warfarin is regarded as the standard treatment for preventing thrombotic events in APS, but the recurrence rate is still high. Dual antiplatelet therapy (DAPT) has been shown to be effective for the prevention of acute coronary syndrome or stroke. The objective of this study was to evaluate the efficacy of DAPT for the prevention of thrombosis recurrence in APS patients with history of arterial thrombosis. Read More

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November 2018

Monitoring disease activity in systemic lupus erythematosus with single-molecule array digital ELISA quantification of serum interferon-α.

Arthritis Rheumatol 2018 Dec 3. Epub 2018 Dec 3.

Sorbonne Université, Inserm UMRS1135, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Assistance Publique-Hôpitaux de Paris, Groupement Hospitalier Pitié-Salpêtrière, Département d'ImmunologieParis, France.

Objectives: No simple or standardized assay is available to quantify interferon-α (IFNα) in routine clinical practice. Single-molecule-array (Simoa) digital enzyme-linked immunosorbent assay (ELISA) technology enables direct IFNα quantification at fg/mL concentrations. This study was undertaken to assess IFNα digital ELISA diagnostic performances to monitor systemic lupus erythematosus (SLE) activity. Read More

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December 2018

Anticoagulating patients with high-risk acquired thrombophilias.

Authors:
Leslie Skeith

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):439-449

Division of Hematology and Hematological Malignancies, Departments of Medicine and Community Health Sciences, University of Calgary, Calgary, Canada; and Clinical Epidemiology Program, Ottawa Hospital Research Institute, Ottawa, Canada.

Antiphospholipid syndrome (APS), heparin-induced thrombocytopenia, and paroxysmal nocturnal hemoglobinuria are 3 acquired thrombophilias that carry a high risk of venous and arterial thromboembolism. Management of these conditions has largely included anticoagulation with a vitamin K antagonist after an initial period of a parenteral anticoagulant, for as long as the thrombotic risk is still present. The available evidence for the use of direct oral anticoagulants (DOACs) is limited and primarily consists of case series and cohort studies, which are summarized in this chapter. Read More

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November 2018

The rheumatology/hematology interface: CAPS and MAS diagnosis and management.

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):313-317

Department of Medicine, Division of Hematology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.

Catastrophic antiphospholipid antibody syndrome (CAPS) and macrophage activation syndrome (MAS) are both life-threatening hematologic disorders that infrequently afflict patients with rheumatologic disease. CAPS is characterized by fulminant multiorgan damage related to small vessel thrombosis in the setting of persistent antiphospholipid antibodies. It can occur in patients with rheumatologic diseases such as systemic lupus erythematosus but can also affect patients who do not have rheumatologic disease. Read More

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November 2018

[Antithrombotics in the management of thrombotic antiphospholipid syndrome].

Rev Med Suisse 2018 Dec;14(630):2198-2201

Service d'angiologie et hémostase, HUG et Faculté de médecine, 1211 Genève 14.

The thrombotic antiphospholipid syndrome is defined by persistent antiphospholipid antibodies and vascular thrombosis. Recommendations for the secondary thrombotic prevention in the thrombotic antiphospholipid syndrome are weak, based on a low-to-moderate quality of evidence. When the initial event leading to diagnosis is a venous thrombosis, the prevention of recurrence of a thrombotic event is based on anticoagulation with a vitamin K antagonist. Read More

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December 2018
2 Reads

Upregulated microRNAs in membranous glomerulonephropathy are associated with significant downregulation of IL6 and MYC mRNAs.

J Cell Physiol 2018 Dec 4. Epub 2018 Dec 4.

Section of Biology and Genetics Giovanni Sichel, Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy.

Membranous glomerulonephropathy (MGN) is a glomerulopathy characterized by subepithelial deposits of immune complexes on the extracapillary side of the glomerular basement membrane. Insertion of C5b-9 (complement membrane-attack complex) into the membrane leads to functional impairment of the glomerular capillary wall. Knowledge of the molecular pathogenesis of MGN is actually scanty. Read More

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December 2018

Anti-RNP/Sm antibodies in patients with systemic lupus erythematosus and its role in thrombosis: a case-control study.

Clin Rheumatol 2018 Dec 4. Epub 2018 Dec 4.

Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, 14000, Mexico City, Mexico.

Objective: To validate the association of thrombotic events with positive lupus anticoagulant (LA) and co-presence of anti-RNP/Sm, as well as the diagnostic accuracy of this combination of antibodies for thrombosis.

Methods: Case-control study of patients with systemic lupus erythematosus (SLE) who presented thrombosis after SLE diagnosis and controls with SLE without thrombosis. Comorbidities, traditional risk factors, clinical variables, and treatment were evaluated. Read More

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December 2018

Post-partum catastrophic antiphospholipid syndrome presenting with shock and digital ischaemia - A diagnostic and management challenge.

J Intensive Care Soc 2018 Nov 19;19(4):357-364. Epub 2018 Mar 19.

Intensive Care Medicine, Nepean Hospital, Kingswood, NSW, Australia.

Catastrophic antiphospholipid syndrome is a rare multisystem autoimmune condition characterised by rapid development of widespread thrombotic disease and subsequent multi-organ failure. It is the most severe complication of antiphospholipid syndrome, carrying significant morbidity and mortality. We report a patient with post-partum catastrophic antiphospholipid syndrome with cardiac, hepatic, renal and cutaneous manifestations. Read More

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November 2018
1 Read

Anti-phospholipid Antibody Syndrome Presenting as Huge Ascites: A Case Report.

Mymensingh Med J 2018 Oct;27(4):883-887

Dr Abed Hussain Khan, Assistant Professor, Department of Internal Medicine, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh.

Antiphospholipid Syndrome (APS) is characterized by arterial, venous or small vessel thromboses and/or pregnancy morbidity in the presence of persistently elevated titers of antiphospholipid antibodies. Since virtually any organ can be involved, the clinical presentation of APS is very varied. Abdominal manifestations are rare but may be life-threatening, and include Budd-Chiari Syndrome. Read More

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October 2018
2 Reads

Addison's disease in antiphospholipid syndrome: a rare complication.

Endocrinol Diabetes Metab Case Rep 2018 Nov 22;2018. Epub 2018 Nov 22.

Endocrinology, Diabetes and Metabolism Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.

Addison's disease (AD) is the most common endocrine manifestation of antiphospholipid syndrome (APS), but it remains a very rare complication of the syndrome. It is caused by adrenal venous thrombosis and consequent hemorrhagic infarction or by spontaneous (without thrombosis) adrenal hemorrhage, usually occurring after surgery or anticoagulant therapy. We present a clinical case of a 36-year-old female patient with a previous diagnosis of APS. Read More

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November 2018
2 Reads

Genome-wide DNA methylation analysis in primary antiphospholipid syndrome neutrophils.

Clin Immunol 2018 Nov 21. Epub 2018 Nov 21.

Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA; Center for Computational Medicine and Bioinformatics, University of Michigan, Ann Arbor, MI, USA. Electronic address:

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thromboembolic events and pregnancy loss. We sought to characterize the DNA methylation profile of primary APS in comparison to healthy controls and individuals with SLE. In primary APS neutrophils compared to controls, 17 hypomethylated and 25 hypermethylated CpG sites were identified. Read More

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November 2018
3 Reads

Differences in plasma fibrin clot composition in patients with thrombotic antiphospholipid syndrome compared with venous thromboembolism.

Sci Rep 2018 Nov 23;8(1):17301. Epub 2018 Nov 23.

Institute of Cardiology, Jagiellonian University Medical College, Krakow, Poland.

The prothrombotic fibrin clot phenotype has been reported in patients with thrombotic antiphospholipid syndrome (APS) and venous thromboembolism (VTE). Protein composition of plasma fibrin clots in APS has not been studied. We evaluated 23 patients with thrombotic APS, 19 with VTE alone, and 20 well-matched controls. Read More

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November 2018
3 Reads

Low extracellular vesicle-associated tissue factor activity in patients with persistent lupus anticoagulant and a history of thrombosis.

Ann Hematol 2018 Nov 22. Epub 2018 Nov 22.

Clinical Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Waehringer Guertel 18-20, A-1090, Vienna, Austria.

Lupus anticoagulants (LA) are a heterogeneous group of antiphospholipid antibodies (aPLAs) that promote thrombosis. Tissue factor (TF)-bearing extracellular vesicles (EVs) might contribute to the prothrombotic state of patients with persistent LA and a history of thrombosis. To investigate if EV-associated TF activity is elevated in a well-defined group of LA-positive patients with a history of thrombosis in comparison to that of healthy controls. Read More

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November 2018
2 Reads

Evaluation of Choroidal Thickness, Choroidal Vascularity Index and Peripapillary Retinal Nerve Fiber Layer in Patients with Juvenile Systemic Lupus Erythematosus.

Lupus 2018 Nov 22:961203318814196. Epub 2018 Nov 22.

2 Department of Ophthalmology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Objective: The aim of this study was to conduct a detailed ophthalmological examination in children with systemic lupus erythematosus (jSLE), including choroidal thickness (ChT), choroidal vascularity index (CVI) and peripapillary retinal nerve fiber layer (RNFL).

Methods: The study included all jSLE patients ( n = 21) diagnosed according to the Systemic Lupus International Collaborating Clinics classification criteria between January 2017 and April 2017, and an age- and gender-matched control group ( n = 21). The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used to assess disease activity. Read More

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November 2018
8 Reads

Prevalence and Clinical Associations of Antiphospholipid Antibodies in Systemic Sclerosis: New Data From a French Cross-Sectional Study, Systematic Review, and Meta-Analysis.

Front Immunol 2018 2;9:2457. Epub 2018 Nov 2.

Univ. Lille, U995, Lille Inflammation Research International Center, Lille, France.

Antiphospholipid antibodies (aPL) can be present in the sera of systemic sclerosis (SSc) patients. This study aimed to determine the prevalence of aPL in a cross-sectional study of SSc patients, to assess their clinical associations, to perform a systematic review of published reports and a meta-analysis to estimate the worldwide prevalence of aPL in SSc. Two-hundred and forty-nine SSc patients were consecutively tested once for lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2glycoprotein I (anti-β2GpI) antibodies. Read More

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November 2018
1 Read

Anticoagulating patients with high-risk acquired thrombophilias.

Authors:
Leslie Skeith

Blood 2018 Nov;132(21):2219-2229

Division of Hematology and Hematological Malignancies, Departments of Medicine and Community Health Sciences, University of Calgary, Calgary, Canada; and Clinical Epidemiology Program, Ottawa Hospital Research Institute, Ottawa, Canada.

Antiphospholipid syndrome (APS), heparin-induced thrombocytopenia, and paroxysmal nocturnal hemoglobinuria are 3 acquired thrombophilias that carry a high risk of venous and arterial thromboembolism. Management of these conditions has largely included anticoagulation with a vitamin K antagonist after an initial period of a parenteral anticoagulant, for as long as the thrombotic risk is still present. The available evidence for the use of direct oral anticoagulants (DOACs) is limited and primarily consists of case series and cohort studies, which are summarized in this chapter. Read More

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November 2018
1 Read

Subclinical atherosclerosis in asymptomatic carriers of persistent antiphospholipid antibodies positivity: A cross-sectional study.

Int J Cardiol 2019 Jan 5;274:1-6. Epub 2018 Jun 5.

Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.

Background: Whereas the relationship between subclinical atherosclerosis and antiphospholipid syndrome (APS) has been widely investigated, little is known about subclinical atherosclerosis in asymptomatic carriers with isolated antiphospholipid antibodies positivity (APP).

Methods: Consecutive APP carriers, APS subjects and matched controls were enrolled. Intima-media thickness of the common carotid artery (CCA-IMT) and of the Bulb (Bulb-IMT) and the prevalence of carotid plaques were assessed in all enrolled subjects. Read More

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January 2019
8 Reads
4.040 Impact Factor

Hughes syndrome (antiphospholipid syndrome) and the nervous system.

Authors:
G Hughes

Lupus 2018 Oct;27(1_suppl):15-17

The London Lupus Centre, London Bridge Hospital, London UK.

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October 2018
1 Read

Antiphospholipid syndrome in obstetrics.

Authors:
M Kemp W Thomas

Lupus 2018 Oct;27(1_suppl):28-31

2 Department of Haematology, Haemophilia & Thrombophilia Centre, Addenbrooke's Hospital, Cambridge, UK.

Antiphospholipid syndrome (APS) covers a spectrum of clinical manifestations ranging from recurrent pregnancy loss and obstetric complications from placental dysfunction through to thrombotic disease. This article will focus on the common manifestations of the pregnancy-related complications of APS. This includes clinical manifestations, diagnosis and management, as general practitioners will need to be able to recognize the disorder and will also have patients under their care receiving treatment for APS. Read More

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October 2018
1 Read

Thrombotic antiphospholipid syndrome.

Authors:
S Sciascia M Radin

Lupus 2018 Oct;27(1_suppl):21-27

1 Center of Research of Immunopathology and Rare Diseases - Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, and SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Italy.

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October 2018
1 Read

Pulmonary thromboendarterectomy is a curative resolution for chronic thromboembolic pulmonary hypertension associated with antiphospholipid syndrome: a retrospective cohort study.

Lupus 2018 Dec;27(14):2206-2214

1 Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and life-threatening condition with poor prognosis in patients with antiphospholipid syndrome (APS). Pulmonary thromboendarterectomy (PTE) is the optimal surgical option for CTEPH.

Objectives: This retrospective cohort study aimed to evaluate the efficacy and risk of PTE in patients with APS-associated CTEPH. Read More

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December 2018
4 Reads

Antiphospholipid antibodies and renal transplant: A systematic review and meta-analysis.

Semin Arthritis Rheum 2018 Oct 19. Epub 2018 Oct 19.

Immune Response and Vascular Disease Unit, CEDOC, NOVA Medical School/Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, Rua Câmara Pestana 6, 1150-082 Lisboa, Portugal.

Objective: To evaluate the effect of antiphospholipid antibodies (aPL) on renal allograft outcome after kidney transplantation.

Methods: A systematic search of EMBASE and PubMed databases from inception to July 2018 was run according to PRISMA guidelines; Peto's odds ratio (OR) for rare events was used for the meta-analysis.

Results: Our inclusion/exclusion criteria were met by 22 cohort studies having different outcomes: allograft thrombosis (n = 9) and thromboprophylaxis (n = 3), allograft loss from any cause (n = 9), allograft malfunction (n = 3), duration (n = 2), glomerular filtration rate at 1 year (n = 3) and allograft rejection (n = 5). Read More

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October 2018
5 Reads

Predictive performance of the competing risk model in screening for preeclampsia.

Am J Obstet Gynecol 2018 Nov 14. Epub 2018 Nov 14.

Institute of Health Research, University of Exeter, Exeter, UK. Electronic address:

Background: The established method of screening for preeclampsia (PE) is to identify risk factors from maternal demographic characteristics and medical history; in the presence of such factors the patient is classified as high-risk and in their absence as low-risk. However, the performance of such approach is poor. We developed a competing risks model which allows combination of maternal factors (age, weight, height, race, parity, personal and family history of PE, chronic hypertension, diabetes mellitus, systemic lupus erythematosus or antiphospholipid syndrome, method of conception and interpregnancy interval), with biomarkers to estimate the individual patient-specific risks of PE requiring delivery before any specified gestation. Read More

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November 2018
4 Reads

JAK2 Specificity and Thrombosis Risk: Potential Role of Antiphospholipid Antibodies.

J Rheumatol 2018 Nov 15. Epub 2018 Nov 15.

Indiana University School of Medicine, Muncie, Indiana; Carnegie Museum - Vertebrate Paleontology, Pittsburgh, Pennsylvania, USA. Address correspondence to Dr. B.M. Rothschild, 789 Bethel Road, Morgantown, West Virginia 26501, USA. E-mail:

Janus kinase (JAK) inhibitors are a relatively new addition to our tools for treatment of rheumatoid arthritis (RA) They are responsible for transduction of more than 38 cytokinases, with diffuse metabolic/immunologic implications. Read More

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November 2018
2 Reads

New insight into antiphospholipid syndrome: antibodies to β2glycoprotein I-domain 5 fail to induce thrombi in rats.

Haematologica 2018 Nov 15. Epub 2018 Nov 15.

Istituto Auxologico Italiano,IRCCS, Milan, Italy;

Clinical studies have reported different diagnostic/predictive values of antibodies to domain 1 or 4/5 of β2glycoproteinI in terms of risk of thrombosis and pregnancy complications in patients with antiphospholipid syndrome. To obtain direct evidence for the pathogenic role of anti-domain 1 or anti-domain 4/5 antibodies, we analysed the in vivo pro-coagulant effect of two groups of 5 serum IgG each reacting selectively with domain 1 or domain 5 in LPS-treated rats. Antibody-induced thrombus formation in mesenteric vessels was followed by intravital microscopy and vascular deposition of β2glycoproteinI, human IgG and C3 was analyzed by immunofluorescence. Read More

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November 2018
5 Reads

Detection of annexin A8 antibodies in serum of patients with antiphospholipid syndrome.

Biochem Med (Zagreb) 2018 Oct;28(3):030703

Institute for Clinical Chemistry, Medical Faculty, University of Cologne, Cologne, Germany.

Introduction: Antibodies specific for annexin A8 (AnxA8) have not been investigated in patients suffering from antiphospholipid syndrome (APS) yet. The aim of this study was to compare the presence of AnxA8 antibodies in serum of APS patients with that of age-matched healthy controls and to investigate whether AnxA8 antibodies are potential biomarkers for APS.

Materials And Methods: We enrolled 22 APS patients and 22 healthy controls in this case-control study. Read More

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October 2018
1 Read

Study of Antiphospholipid Antibodies in Patients with Arterial Hypertension.

Med Sci (Basel) 2018 Nov 13;6(4). Epub 2018 Nov 13.

Laboratory of Microbiology, Democritus University of Thrace, University General Hospital of Alexandroupolis, Dragana Campus, 68100 Alexandroupolis, Greece.

Antiphospholipid syndrome (APS) is a multifactorial, autoantibody-mediated disease. Antiphospholipid antibodies (aPL) directed against negatively charged phospholipids or various combinations of phospholipid-binding proteins seem to be an independent pathogenic factor that plays a critical role in APS. Unfortunately, their role in hypertension is not fully elucidated. Read More

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November 2018
1 Read

Bilateral central retinal artery occlusion from catastrophic antiphospholipid syndrome.

BMJ Case Rep 2018 Nov 12;2018. Epub 2018 Nov 12.

Department of Internal Medicine, John H Stroger Jr Hospital of Cook County, Chicago, Illinois, USA.

A 23-year-old woman with history of systemic lupus erythematous presented with dizziness and headache and was admitted for the stroke workup. During her stay, she had sudden painless loss of vision in her right eye consistent with central retinal artery occlusion (CRAO). Ocular massage and paracentesis were attempted without success to resume the flow. Read More

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November 2018
6 Reads

Cofactor-Independent Antiphospholipid Antibodies: Implications for Pathogenesis, Diagnosis, and Treatment of Antiphospholipid Syndrome.

Hamostaseologie 2018 Nov 12. Epub 2018 Nov 12.

Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany.

The antiphospholipid syndrome (APS) has occupied haemostaseologists, rheumatologists and obstetricians since its initial description 35 years ago. Its name has been coined because of the antibodies against phospholipids which were the common property of affected patients. In particular, the pathogenesis of APS has been intensively studied after the early discovery that it was possible to induce the clinical manifestations in animals by transfer of antiphospholipid antibodies (aPL). Read More

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November 2018
1 Read

Self-reported disease severity in women with systemic lupus erythematosus.

Rheumatol Int 2018 Nov 10. Epub 2018 Nov 10.

Internal Medicine Department, Carol Davila UMF, Dionisie Lupu Street 37, 020022, Bucharest S2, Romania.

Systemic lupus erythematosus (SLE), pathology with net feminine predominance, is one of the most complex autoimmune diseases and has major impact on patients' life. The aim is to identify patient and disease-related factors associated with self-perceived disease severity in female SLE patients. This cross-sectional study enrolled 73 women fulfilling the 2012 Systemic Lupus International Collaborating Clinic (SLICC) criteria. Read More

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November 2018
3 Reads

Relationship between cerebral microbleeds and white matter MR hyperintensities in systemic lupus erythematosus: a retrospective observational study.

Neuroradiology 2018 Nov 10. Epub 2018 Nov 10.

Department of Neuropsychiatry, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.

Purpose: White matter hyperintensities (WMH) and cerebral microbleeds (CMBs) are known to be associated with small vessel diseases (SVD) and neuroinflammation. The purpose was to investigate the relationship between CMBs and WMH in patients with systemic lupus erythematosus (SLE).

Methods: Thirty-one SLE patients with WMH and 27 SLE patients with normal brain MRI were compared. Read More

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November 2018
3 Reads

Pathogenesis of antiphospholipid syndrome: recent insights and emerging concepts.

Expert Rev Clin Immunol 2018 Nov 9. Epub 2018 Nov 9.

a Institute of Clinical Chemistry and Laboratory Medicine , University Medical Center of the Johannes Gutenberg University , Mainz , Germany.

Introduction: Even though our understanding of the antiphospholipid syndrome (APS) has improved tremendously over the last decades, we are still not in a position to replace symptomatic anticoagulation by pathogenesis based causal treatments. Areas covered: Recent years have provided further insights into pathogenetically relevant mechanisms. These include a differentiation of pathogenic subtypes of antiphospholipid antibodies (aPL), novel mechanisms modulating disease activity, e. Read More

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November 2018
2 Reads

Primary Nonbacterial Thrombotic Endocarditis Presenting with Bowel Infarction Secondary to Superior Mesenteric Artery Embolism.

Methodist Debakey Cardiovasc J 2018 Jul-Sep;14(3):228-231

UTMB SCHOOL OF MEDICINE, GALVESTON, TEXAS.

Nonbacterial thrombotic endocarditis (NBTE) is a rare antemortem diagnosis that is commonly associated with hypercoagulable states such as advanced malignancies, disseminated intravascular coagulation, and autoimmune diseases such as antiphospholipid syndrome and systemic lupus erythematosus. We present a case of a previously healthy 42-year-old man who presented with small bowel infarction caused by embolic occlusion of the superior mesenteric artery and was subsequently diagnosed with NBTE. Despite thorough investigation, efforts to find an underlying cause failed to reveal any associated systemic illnesses. Read More

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November 2018
1 Read

Guidelines for biomarkers in autoimmune rheumatic diseases - evidence based analysis.

Autoimmun Rev 2018 Nov 5. Epub 2018 Nov 5.

Pathophysiology Department, General Hospital of Athens "Laiko", Medical School, National and Kapodistrian University of Athens, Greece.

Autoimmune rheumatic diseases are characterised by an abnormal immune system response, complement activation, cytokines dysregulation and inflammation. In last years, despite many progresses in managing these patients, it has been shown that clinical remission is reached in less than 50% of patients and a personalised and tailored therapeutic approach is still lacking resulting in a significant gap between guidelines and real-world practice. In this context, the need for biomarkers facilitating early diagnosis and profiling those individuals at the highest risk for a poor outcome has become of crucial interest. Read More

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November 2018
7 Reads

Adrenal Insufficiency in Systematic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS): A Systematic Review.

Autoimmun Rev 2018 Nov 5. Epub 2018 Nov 5.

Department of Pediatrics, Yonsei University College of Medicine, Seoul, Republic of Korea; Yonsei University College of Medicine, Department of Pediatric Nephrology, Severance Children's Hospital, Seoul, Republic of Korea. Electronic address:

Background: Adrenal insufficiency (AI) is associated with high morbidity and mortality. The aim of this systematic review was to enhance diagnostic approaches and summarize therapeutic strategies in the management of AI in patients with an underlying prior disease history.

Methods: A literature search of PubMed and EMBASE databases was performed and 91 publications containing 105 cases were included for the final analysis. Read More

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November 2018
8 Reads

Venous thromboembolic events during warm autoimmune hemolytic anemia.

PLoS One 2018 8;13(11):e0207218. Epub 2018 Nov 8.

Department of Internal Medicine and Clinical Immunology, Constitutive Referral Center for Autoimmune Cytopenias, University Hospital, Dijon, France.

Thrombotic manifestations are a hallmark of many auto-immune diseases (AID), specially of warm autoimmune hemolytic anemia (wAIHA), as 15 to 33% of adults with wAIHA experience venous thromboembolic events (VTE). However, beyond the presence of positive antiphospholipid antibodies and splenectomy, risk factors for developing a VTE during wAIHA have not been clearly identified. The aim of this retrospective study was to characterize VTEs during wAIHA and to identify risk factors for VTE. Read More

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November 2018
5 Reads

PEGylated Domain I of Beta-2-Glycoprotein I Inhibits the Binding, Coagulopathic, and Thrombogenic Properties of IgG From Patients With the Antiphospholipid Syndrome.

Front Immunol 2018 22;9:2413. Epub 2018 Oct 22.

Division of Medicine, Centre for Rheumatology Research, University College London, London, United Kingdom.

APS is an autoimmune disease in which antiphospholipid antibodies (aPL) cause vascular thrombosis and pregnancy morbidity. In patients with APS, aPL exert pathogenic actions by binding serum beta-2-glycoprotein I (β2GPI) via its N-terminal domain I (DI). We previously showed that bacterially-expressed recombinant DI inhibits biological actions of IgG derived from serum of patients with APS (APS-IgG). Read More

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October 2018
9 Reads

Zonal cortical scarring and tubular thyroidization in kidney biopsies of patients with SLE-histologic indicator for antiphospholipid antibodies.

Lupus 2018 Dec 7;27(14):2236-2244. Epub 2018 Nov 7.

2 Department of Pathology, Division of Renal and Transplant Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.

Antiphospholipid antibody syndrome (APS) is an acquired prothrombotic autoimmune disease caused by the presence of antibodies against anionic phospholipids or plasma proteins bound to phospholipids on cell membranes. It can be a primary disease or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE). Laboratory testing for antiphospholipid antibodies (aPL) may be only transiently positive, so APS could be missed until a catastrophic thrombotic episode or pregnancy morbidity occurs. Read More

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December 2018
6 Reads

Antiphospholipid syndrome: state of the art on clinical practice guidelines.

RMD Open 2018 18;4(Suppl 1):e000785. Epub 2018 Oct 18.

Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy.

Antiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies. This review aims to identify existing clinical practice guidelines (CPG) as part of the ERN ReCONNET project, aimed at evaluating existing CPGs or recommendations in rare and complex diseases. Seventeen papers providing important data were identified; however, the literature search highlighted the scarceness of reliable clinical data to develop CPGs. Read More

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October 2018
15 Reads

Intrauterine fetal deaths related to antiphospholipid syndrome: a descriptive study of 65 women.

Arthritis Res Ther 2018 Nov 6;20(1):249. Epub 2018 Nov 6.

AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'île de France, 27 Rue du Faubourg Saint Jacques, 75014, Paris, France.

Objective: Although one of the three obstetric manifestations of antiphospholipid syndrome (APS) is intrauterine fetal death (IUFD), little is known about it in this context. We report the first large series of patients with APS and IUFD.

Methods: We retrospectively analyzed the history and clinical data of women at four French hospitals. Read More

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November 2018
4 Reads

Hydroxychloroquine partially prevents endothelial dysfunction induced by anti-beta-2-GPI antibodies in an in vivo mouse model of antiphospholipid syndrome.

PLoS One 2018 6;13(11):e0206814. Epub 2018 Nov 6.

UMR CNRS 6015-INSERM U1083, MITOVASC Institute, Université d'Angers, Faculté de Médecine, Bâtiment IRIS 2, Angers, France.

Background: Antiphospholipid syndrome is associated with endothelial dysfunction, which leads to thrombosis and early atheroma. Given that hydroxychloroquine has anti-thrombotic properties in lupus, we hypothesized that it could reduce endothelial dysfunction in an animal model of antiphospholipid syndrome. We evaluated the effect of hydroxychloroquine in preventing endothelial dysfunction in a mouse model of antiphospholipid syndrome. Read More

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November 2018
8 Reads

[Clinical features of patients with primary Sjögren's syndrome complicated with venous thrombosis].

Authors:
Y F Zhang Y W Lu

Zhonghua Yi Xue Za Zhi 2018 Oct;98(39):3197-3199

To analyze the clinical characteristics of primary Sjögren's syndrome (pSS) with venous system thrombosis (VT), and to improve the understanding of the disease. The clinical and laboratory characteristics of 16 cases of pSS with VT were analyzed retrospectively. Among 16 cases, 12 cases was women, 2 case was men, age between 45 and 71. Read More

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October 2018
1 Read

[Clinical study of maternal and infant outcomes in patients with lupus nephritis complicated with pregnancy].

Zhonghua Yi Xue Za Zhi 2018 Oct;98(37):2982-2986

Department of Rheumatology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China.

To investigate the outcomes and associated factors for adverse pregnancy outcomes (APO) in pregnant patients with lupus nephritis (LN). The clinical data of 139 LN pregnant patients from from 2009 to 2017 in the First Affiliated Hospital of Sun Yat-sen University were analyzed retrospectively. Totally, 105 LN were diagnosed before pregnancy and 34 were newly diagnosed during pregnancy. Read More

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October 2018
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Multifocal avascular osteonecrosis despite appropriate anticoagulation therapy in a patient with systemic lupus erythematosus and antiphospholipid syndrome.

BMJ Case Rep 2018 Nov 1;2018. Epub 2018 Nov 1.

Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, University of Turin and S. Giovanni Bosco Hospital, Turin, Italy.

Multifocal avascular osteonecrosis (AON) is a serious manifestation of systemic lupus erythematosus (SLE). Prothrombotic factors, especially antiphospholipid antibodies (aPL), have been associated with the development of AON; therefore, attenuating the procoagulant state while balancing the haemorrhagic risks might have a rationale when managing this condition. We report a case of a 37-year-old patient with SLE, treated with low doses of corticosteroids and immunosuppressive therapy, who was started on vitamin K antagonist following an episode of deep vein thrombosis while having persistent positivity for aPL. Read More

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November 2018
4 Reads

Natural joints: Boundary lubrication and antiphospholipid syndrome (APS).

Biosystems 2018 Oct 31. Epub 2018 Oct 31.

Faculty of Mathematics, Physics and Technical Sciences, Kazimierz Wielki University, Chodkiewicza 30, 85-867, Bydgoszcz, Poland.

The paper shows that osteoporosis (OA) changes the SF content and the lipid profile substantially. To estimate the implication of the lipid environment in case the articular cartilage (AC) changes, we measured friction coefficient normal samples, with early and late stages of (OA). During joint inflammation and osteoarthritis, enzymatically activated β-Glycoprotein I is transformed into antibody conformation. Read More

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October 2018
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Tulip piercing the aorta: a rare case of IVC filter aortic perforation and obstruction.

J Surg Case Rep 2018 Oct 29;2018(10):rjy280. Epub 2018 Oct 29.

Section of Vascular Surgery, Department of Surgery, Washington University, St. Louis, MO, USA.

Prolonged implantation of inferior vena cava (IVC) filters can lead to significant morbidity. We present a 25-year-old man with antiphospholipid syndrome, lower extremity deep vein thrombosis, and subsequent Gunther-Tulip IVC filter placement. More than 10 years following IVC filter placement he developed progressive abdominal and back pains. Read More

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October 2018
5 Reads

Antiphospholipid antibodies associated with nodal marginal zone lymphoma and its progression to diffuse large B-cell lymphoma-A case report.

Pathol Res Pract 2018 Oct 23. Epub 2018 Oct 23.

Department of Pathology, University of Rijeka, Faculty of Medicine, Croatia.

An association between autoimmune events, as well as the development of antiphospholipid (aPL) antibodies and lymphoproliferative disorders is well recognized. We present the patient with coagulation abnormalities and non-Hodgkin lymphoma (NHL), primarily diagnosed as nodal marginal zone B-cell lymphoma (NMZL), and in relapse as diffuse large B-cell lymphoma (DLBCL). In the follow-up period, the patient simultaneously developed different aPL antibodies. Read More

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October 2018
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Prevalence and correlates of thrombosis in adults with immune thrombocytopenia: An NIS study.

Thromb Res 2018 Dec 24;172:80-85. Epub 2018 Oct 24.

Center for Research on Health Care Data Center, University of Pittsburgh, Pittsburgh, PA, United States of America; Department of Medicine, University of Pittsburgh, Pittsburgh, PA, United States of America; Division of General Internal Medicine, University of Pittsburgh, Pittsburgh, PA, United States of America; Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA, United States of America.

Background: Immune thrombocytopenia (ITP) is increasingly recognized as a thrombophilic disorder. However, no further investigation of risk factors has been conducted to date. This study evaluated classic and disease-specific correlates of thrombosis among ITP patients. Read More

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December 2018
3 Reads