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Antiphospholipid Syndrome

Gokhan Sargin, Taskin Senturk, Songul Cildag
A 69-year-old female patient was admitted to our clinic with photosensitivity, symmetric erosive polyarthritis, and cutaneous vasculitis of lower extremities. Rhupus syndrome was diagnosed, and Chiari's network in the right atrium and interatrial septum patent foramen ovale was achieved on transthoracic and transesophageal echocardiography. If it is thought that increased prevalence of antiphospholipid antibodies in patients with rhupus, this congenital remnant is important for the thrombosis risk, cardiac event, and stroke...
January 2018: Journal of Family Medicine and Primary Care
Anne F M Jansen, Ruud P H Raijmakers, Stephan P Keijmel, Renate G van der Molen, Gerald M Vervoort, Jos W M van der Meer, Marcel van Deuren, Chantal P Bleeker-Rovers
Q fever infection can lead to chronic Q fever, a potentially lethal disease occurring in 1-5% of patients infected with Coxiella burnetii, characterized by the persistence of this intracellular bacterium. It usually presents as endocarditis, infected vascular aneurysms, or infected vascular prostheses. This systematic review of the literature discusses the various autoimmune syndromes and B-cell dyscrasias in acute and chronic Q fever patients, that may interfere with or impede recognition and diagnosis of Q fever...
June 13, 2018: European Journal of Internal Medicine
Nathaniel Harris, Amanda Eudy, Megan Clowse
OBJECTIVES: While increased rheumatic disease activity during pregnancy has been associated with adverse pregnancy outcomes, this activity is typically assessed by the physician. Little is known, however, about the association between patient-reported measures of disease activity and pregnancy outcomes. METHODS: Univariate and multivariable regression models were used to assess the relationship between patient and physician-reported measures of disease activity and adverse pregnancy outcomes in 225 patients with lupus or rheumatoid arthritis (RA) enrolled in a prospective registry at a single academic center from 2008-2016...
June 15, 2018: Arthritis Care & Research
Maria G Tektonidou
Kidney damage is a well-recognized complication of the antiphospholipid syndrome (APS), either primary or systemic lupus erythematosus (SLE)-associated APS. Kidney involvement in APS involves a variety of manifestations, such as renal artery thrombosis or stenosis, renal vein thrombosis, allograft loss due to thrombosis after kidney transplantation, and injury to the renal microvasculature, also known as APS nephropathy. Biopsy in patients with APS nephropathy includes acute thrombotic microangiopathy lesions and chronic intrarenal vascular lesions such as interlobular fibrous intimal hyperplasia, arterial and arteriolar recanalizing thrombosis, fibrous arterial occlusion, and focal cortical atrophy...
2018: Frontiers in Immunology
Romy M W Kremers, Stéphane Zuily, Hilde Kelchtermans, Tessa C Peters, Saartje Bloemen, Véronique Regnault, H Coenraad Hemker, Philip G de Groot, Denis Wahl, Bas de Laat
Antiphospholipid syndrome (APS) is a condition in which the presence of antibodies against phospholipid-binding proteins is associated with thrombophilia and/or pregnancy morbidity. Although antiphospholipid antibodies have anticoagulant characteristics in vitro, they are associated with thromboembolic complications. Thrombin generation (TG) is a sensitive global test of coagulation, and elevated TG is associated with thrombosis. Increased TG can be caused by increased prothrombin conversion, decreased thrombin inactivation, or a combination of both...
June 12, 2018: Blood Advances
Ya-Hsuan Chao, Der-Yuan Chen, Joung-Liang Lan, Kuo-Tung Tang, Chi-Chien Lin
DNA vaccines have recently emerged as a therapeutic agent for treating autoimmune diseases, such as multiple sclerosis. Antiphospholipid antibody syndrome (APS) is an autoimmune disease characterized by β2-glycoprotein I (β2-GPI)-targeting antiphospholipid antibodies (APAs) and vascular thrombosis or obstetrical complications. To examine the therapeutic potential of a β2-GPI DNA vaccine, we administered a vaccine mixed with FK506 as an adjuvant to a mouse model of obstetric APS. First, the pCMV3-β2-GPI DNA vaccine, which encodes the full-length human β2-GPI gene, was constructed...
2018: PloS One
Yoshimitsu Kuwabara, Akira Katayama, Sachiko Kurihara, Hideo Orimo, Toshiyuki Takeshita
Immunoproteomic analysis was performed to identify unknown, pathology-related molecules in patients with seronegative (SN) obstetric antiphospholipid syndrome (APS) who clinically satisfied the diagnostic criteria for APS, but not the serological criteria. We collected peripheral blood from 13 SN-APS outpatients with known thrombotic predisposition, 13 with no known thrombotic predisposition, and four multiparous women with no history of miscarriage (control). Plasma proteins from volunteers were purified and used as plasma protein antigens...
2018: PloS One
Pier Luigi Meroni, Maria Orietta Borghi, Claudia Grossi, Cecilia Beatrice Chighizola, Paolo Durigutto, Francesco Tedesco
Recurrent thrombosis and miscarriages are the main clinical manifestations of antiphospholipid syndrome (APS). Although most patients display both clinical signs, some patients can have isolated vascular or obstetric variants. Emerging data raise the question of whether obstetric and vascular APS are the same or different diseases. An important difference between the two conditions is that a thrombophilic state is a common feature in vascular APS, whereas clot occlusions of the decidual spiral arteries are seldom observed in obstetric APS, and infarctions are found in only one-third of APS placentae...
June 11, 2018: Nature Reviews. Rheumatology
Debora N Setoue, Ana C Pitta, Fernanda J Fiorot, Mariana M Nastri, Glaucia V Novak, Beatriz C Molinari, Juliana C Oliveira, Natali W Gormezano, Ana P Sakamoto, Maria T Terreri, Rosa M Pereira, Claudia Saad-Magalhães, Adriana M Sallum, Katia Kozu, Melissa M Fraga, Daniela P Piotto, Gleice Clemente, Roberto Marini, Hugo R Gomes, Carlos N Rabelo-Junior, Marta M Felix, Maria C Ribeiro, Rozana G Almeida, Ana P Assad, Silvana B Sacchetti, Leandra C Barros, Eloisa Bonfá, Clovis A Silva
OBJECTIVE: To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus(cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. METHODS: A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD...
June 7, 2018: Autoimmunity Reviews
Cristina Belizna, Ljudmila Stojanovitch, Jan Willem Cohen Tervaert, Céline Fassot, Daniel Henrion, Laurent Loufrani, Gyorgy Nagy, Christian Muchardt, Milena Hasan, Marie Noelle Ungeheuer, Laurent Arnaud, Jaume Alijotas Reig, Enrique Esteve Valverde, Ferdinando Nicoletti, Patrick Saulnier, Alban Godon, Pascal Reynier, Jean Marie Chrétien, Laura Damian, Loukman Omarjee, Guillaume Mahé, Marc Antoine Pistorius, Pier Luigi Meroni, Katrien Devreese
Primary antiphospholipid syndrome (PAPS) and antiphospholipid syndrome associated to lupus (SAPS) have several overlapping characteristics. As systemic manifestations are also reported in patients with PAPS, and as a subgroup of PAPS patients could evaluate to a SAPS, the differentiation between the two types of APS could be performed based on the clinical experience of the medical teams and is related to a variety of clinical, biological, histological and genetic features. Several data are available in the literature with respect to the identification of distinctive features between these two entities...
June 6, 2018: Autoimmunity Reviews
Aleksandra Rymarz, Stanisław Niemczyk
BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome (APS) involving many organs and leading to their insufficiency. The pathogenesis of CAPS is associated with the presence of antiphospholipid antibodies (aPL). Typical therapy includes anticoagulation, glucocorticoids, therapeutic plasma exchanges and/or intravenous immunoglobulin. Despite this aggressive treatment, the mortality rate of 37% is still high. Novel therapeutic agents are required...
June 8, 2018: BMC Nephrology
Thomas B Martins, Nahla Heikal, Jason Miller, Rohan Willis, Robert L Schmidt, Anne E Tebo
BACKGROUND: We assessed the performance characteristics and correlations of the traditional enzyme-linked immunosorbent assay (ELISA) and chemiluminescence immunoassay (CIA) for detecting IgG and IgM antibodies to cardiolipin (aCL) and beta2 glycoprotein (anti-β2 GPI) antibodies in patients under routine evaluation for APS. METHODS: Patients (n = 216) referred to ARUP Laboratories for lupus anticoagulant (LAC) and/or aCL or anti-β2 GPI IgG/IgM antibodies evaluation were assessed by ELISA and CIA methods...
June 5, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
Zbigniew Zdrojewski
Recognizing the antiphospholipid syndrome (APS) in patients with lupus (SLE) can be difficult and therefore underestimated. Detection of antiphospholipid antibodies (aPL) in each patient with SLE should be done as a rule. Introduction of the new classification criteria of this syndrome will certainly improve the recognition of APS in the future. The Sapporo APS classification criteria (1998) were replaced by the Sydney criteria in 2006. Based on the most recent criteria, classification with APS requires one clinical and one laboratory manifestation...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Nikolaos Melas, Amil Haji Younes, Robert Lindberg, Peter Magnusson
Mesenteric ischemia and antiphospholipid syndrome is a rare combination but should be suspected as a differential diagnosis. This may be presented as diffuse abdominal pain typically after food intake, diarrhea, and weight loss. Early recognition is warranted, and nutrition, stenting, and anticoagulant treatments are indicated.
June 2018: Clinical Case Reports
Arturo J Bonnin, Charles DeBrosse, Terri Moncrief, G Wendell Richmond
Background: Angioedema secondary to acquired C1 inhibitor deficiency (AAE) is a rare disease. It usually is associated with lymphoproliferative disorders. We present a case of AAE in a patient with antiphospholipid syndrome (APS), a non-Hodgkin lymphoproliferative disorder (NHL) with undetectable levels of C2, C4, and an undetectable CH50. The co-existence of AAE, APS, and NHL, with an undetectable C2 level, to the best of our knowledge, has never before reported together in the same patient...
2018: Allergy, Asthma, and Clinical Immunology
Pravesh Kumar Bundhun, Mohammad Zafooruddin Sani Soogund, Feng Huang
BACKGROUND: We aimed to systematically compare arterial/venous thrombosis, fetal loss and stillbirth in pregnant women with systemic lupus erythematosus (SLE), primary anti-phospholipid syndrome (PAPS) and secondary anti-phospholipid syndrome (SAPS). METHODS: Online databases were carefully searched for relevant publications comparing SLE with PAPS and/or SAPS in pregnancy. Studies were included if: they compared SLE with APS [SLE versus PAPS or SLE versus SAPS or SLE versus PAPS and SAPS respectively] in pregnant women; and they reported specific adverse outcomes as their clinical endpoints including arterial/venous thrombosis, fetal loss and stillbirth...
June 7, 2018: BMC Pregnancy and Childbirth
Joanna Natorska, Magdalena Celińska-Löwenhoff, Anetta I Undas
BACKGROUND: Growing evidence suggests that activation of blood coagulation exerts protective functions during inflammation. However, it has been hypothesized that dysregulated immunothrombosis may lead to venous thromboembolism (VTE). Antinuclear antibodies (ANAs) are considered to promote the thrombotic tendency but there have been no reports on the association between ANAs and VTE. OBJECTIVES: The objective of this study was to investigate if the presence of ANAs is associated with VTE...
June 7, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Lucas L van den Hoogen, Marzia Rossato, Ana P Lopes, Aridaman Pandit, Cornelis P J Bekker, Ruth D E Fritsch-Stork, Joel A G van Roon, Timothy R D J Radstake
Objective: To investigate miRNA expression in relation to transcriptomic changes in plasmacytoid dendritic cells (pDCs) in SLE and APS. pDCs are major producers of IFNα in SLE and APS, and miRNAs are emerging as regulators of pDC activation. Methods: miRNA and mRNA expression were measured by OpenArray and RNA-sequencing in pDCs of SLE, SLE + APS (APS secondary to SLE) and primary APS (PAPS) patients. The miRNA profile of patients was compared with the miRNA pattern of TLR7-activated pDCs...
June 4, 2018: Rheumatology
Warsame Ibrahim, Hunter Nakia, Miller Stephen, Spiess Bruce, Whitson Bryan, Perez William
Anticoagulation for cardiopulmonary bypass (CPB) is required to prevent acute disseminated intravascular coagulation and clot formation within the bypass circuit. Unfractionated heparin is the standard anticoagulant for CPB due to its many advantages and long history of successful use. However, heparin has the unique drawback of triggering Heparin-PF4 (PF4) antibodies potentially leading to heparin-induced thrombocytopenia (HIT). We have limited data regarding reformation of antibodies if a patient has had a prior (remote) antibody production or full HIT...
June 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
Miklós Pólos, Attila Kovács, Endre Németh, Béla Merkely
Antiphospholipid syndrome is an autoimmune disorder with frequent clinical manifestation of arterial, venous or small vessel thromboembolic events and/or foetal morbidity. Hereby, we present a case report of a patient with a large arterial thrombus originating from the ostium of the right coronary artery accompanied by multiple systemic embolization and right ventricular failure. As a bridge-to-recovery strategy, an extracorporeal right ventricular assist device was successfully applied, representing an effective first choice option in selected patients with isolated acute right heart failure...
June 3, 2018: European Journal of Cardio-thoracic Surgery
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