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secondary antiphospholipid antibody syndrome

K T El Hadidi, B M Medhat, N M Abdel Baki, H Abdel Kafy, W Abdelrahaman, A Y Yousri, D H Attia, M Eissa, D El Dessouki, I Elgazzar, F T Elgengehy, N El Ghobashy, H El Hadary, G El Mardenly, H El Naggar, A M El Nahas, R M El Refai, H Allah El Rwiny, R M Elsman, M Galal, S Ghoniem, L A Maged, S M Sally, H Naji, S Saad, M Shaaban, M Sharaf, N Sobhy, R M Soliman, T S El Hadidi
Introduction Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can vary among different ethnic and racial groups. Objective The objective of this paper is to study the prevalence of various manifestations of SLE in a sample of the Egyptian population. Patients and methods Information in this study was derived from the medical records of SLE patients who sought medical advice at a private clinic in Cairo from January 1980 to June 2016. Results This study included 1109 SLE patients, of whom 114 (10...
January 1, 2018: Lupus
Hui Shi, Hui Zheng, Yu-Feng Yin, Qiong-Yi Hu, Jia-Lin Teng, Yue Sun, Hong-Lei Liu, Xiao-Bing Cheng, Jun-Na Ye, Yu-Tong Su, Xin-Yao Wu, Jin-Feng Zhou, Gary L Norman, Hui-Yun Gong, Xin-Ming Shi, Yi-Bing Peng, Xue-Feng Wang, Cheng-De Yang
BACKGROUND: The aim of the study was to determine the prevalence and clinical associations of antiphosphatidylserine/prothrombin antibodies (aPS/PT) with thrombosis and pregnancy loss in Chinese patients with antiphospholipid syndrome (APS) and seronegative APS (SNAPS). METHODS: One hundred and eighty six Chinese patients with APS (67 primary, 119 secondary), 48 with SNAPS, 176 disease controls (79 systemic lupus erythematosus [SLE], 29 Sjogren's syndrome [SS], 30 ankylosing spondylitis [AS], 38 rheumatoid arthritis [RA]) and 90 healthy donors were examined...
November 22, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
Jovica Saponjski, Ljudmila Stojanovich, Jelena Saponjski, Milorad Mirilovic, Dusan Saponjski
Antiphospholipid syndrome (APS) is an autoimmune disease defined by accelerated atherosclerosis, arterial and venous thrombosis, fetal loss, and the presence of antiphospholipid antibodies (aPL) in the serum and which leads to the occurrence of various vascular events. Nonspecific abdominal pain can be one of the symptoms due to changes on visceral blood vessels. The goal of our work is to show the results we obtained in multidetector computed tomography (MDCT) angiography examination of visceral arteries, comparing patients with primary antiphospholipid syndrome (PAPS) and secondary antiphospholipid syndrome (SAPS) with control group...
December 2017: Immunologic Research
William E Winter, Sherri D Flax, Neil S Harris
Primary hemostasis begins with endothelial injury. VWF, produced by endothelial cells, binds to platelets and links them to subendothelial collagen. Platelet-derived ADP and thromboxane activate non-adhered platelets via their GPIIb/IIIa receptors, allowing these platelets to participate in platelet aggregation. Secondary hemostasis is initiated with the binding of factor VII to extravascular tissue factor (TF). Factors II, VII, IX and X are vitamin K-dependent factors. The role of vitamin K is to assist in the addition of gamma carboxylate groups to glutamic acids in the "GLA" domains of these factors...
November 8, 2017: Laboratory Medicine
Mirjana Bećarević
Antiphospholipid syndrome (APS) is an autoimmune disease that is characterized by arterial and/or venous thrombosis and/or recurrent pregnancy losses. Obstetric APS (OAPS) is considered as a distinct entity from vascular APS (VAPS). In the absence of any additional disease, APS is designated as primary (PAPS), while the term secondary APS (SAPS) is used when other diseases are associated. Catastrophic APS (CAPS) is characterized by the rapid development of multiple thrombosis in various vital organs. The presence of antiphospholipid antibodies (aPL Abs) is considered as a laboratory criterion for APS diagnosis...
November 2017: Journal of Thrombosis and Thrombolysis
Malgorzata M Bala, Magdalena Celinska-Lowenhoff, Wojciech Szot, Agnieszka Padjas, Mateusz Kaczmarczyk, Mateusz J Swierz, Anetta Undas
BACKGROUND: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial or venous thrombosis (or both) and/or pregnancy morbidity in association with the presence of antiphospholipid antibodies. The prevalence is estimated at 40 to 50 cases per 100,000 people. The most common sites of thrombosis are cerebral arteries and deep veins of the lower limbs. People with a definite APS diagnosis have an increased lifetime risk of recurrent thrombotic events. OBJECTIVES: To assess the effects of antiplatelet or anticoagulant agents, or both, for the secondary prevention of recurrent thrombosis, particularly ischemic stroke, in people with antiphospholipid syndrome...
October 2, 2017: Cochrane Database of Systematic Reviews
Rukma Parthvi, Rutuja R Sikachi, Abhinav Agrawal, Ajay Adial, Abhinav Vulisha, Sameer Khanijo, Arunabh Talwar
Antiphospholipid (aPL) antibodies are antibodies specific for anionic phospholipids. They are immunoglobulins that attack phospholipids, phospholipid-binding proteins, or phospholipid-protein complexes and are detected in anticardiolipin and lupus anticoagulant assays. aPL antibodies are often associated with antiphospholipid syndrome (APS) which can be idiopathic or from secondary causes such as systemic lupus erythematosus (SLE), infection or drugs. They have also been shown to be associated with Pulmonary Hypertension...
August 2017: Intractable & Rare Diseases Research
Carlos A Hinojosa, Javier E Anaya-Ayala, Karla Bermudez-Serrato, Ramón García-Alva, Hugo Laparra-Escareno, Adriana Torres-Machorro, Rene Lizola
OBJECTIVE: The association of antiphospholipid antibody syndrome (APS) and hypercoagulability is well known. Arterial compromise leading to ischemia of organs and/or limbs in patients with APS is uncommon, frequently unrecognized, and rarely described. We evaluated our institutional experience. METHODS: Retrospective review was conducted. From August 2007 to September 2016, 807 patients with diagnosis of APS were managed in our Institution. Patients with primary and secondary APS who required interventions were examined...
November 2017: Vascular and Endovascular Surgery
M A Islam, F Alam, S H Gan, T H Sasongko, W S Wan Ghazali, K K Wong
BACKGROUND: Antiphospholipid syndrome (APS) is an autoimmune disorder characterised by thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPLs) based on the Sydney criteria. We aimed to explore the clinico-laboratory features and treatment strategies of APS patients retrospectively. METHODOLOGY: The medical records of APS patients registered under Hospital Universiti Sains Malaysia (Kelantan state) between 2000 and 2015 were reviewed...
August 2017: Malaysian Journal of Pathology
D Erkan, O Unlu, S Sciascia, H M Belmont, D Ware Branch, M J Cuadrado, E Gonzalez, J S Knight, I Uthman, R Willis, Z Zhang, D Wahl, S Zuily, M G Tektonidou
Objective The objective of this study was to determine the efficacy of hydroxychloroquine (HCQ) in the primary thrombosis prevention of antiphospholipid antibody (aPL)-positive patients with no other systemic autoimmune diseases. Methods Under the auspices of Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking, a multicenter, international, randomized controlled trial (RCT) was initiated, in which persistently aPL-positive but thrombosis-free patients without systemic autoimmune diseases were randomized to receive HCQ or no treatment in addition to their standard regimen...
January 1, 2017: Lupus
Thelma Skare, Leandro Picelli, Thiago Alberto G Dos Santos, Renato Nisihara
The objective of the study is to study the positivity of Coombs test or direct antiglobulin test (DAT) in systemic lupus erythematosus (SLE) patients and its relationship with disease's clinical and serological profile. Retrospective study of 373 SLE patients seen at single Rheumatology Unit. Epidemiological data (age, gender, age at disease onset, auto declared ethnic background and tobacco use), clinical (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia and arthritis), and serological profile (anti ds DNA, anti Ro/SS-A; anti La/SS-B, Anti RNP, Anti Sm, aCl (anticardiolipin) IgG, aCl Ig M, LA or lupus anticoagulant, rheumatoid factor and direct Coombs) were collected...
September 2017: Clinical Rheumatology
Nicolas Belhomme, Marine Le Noir De Carlan, Alain Lescoat, Thomas Le Gallou, Florence Rouget, Philippe Loget, Patrick Jego
AIM: The objectives were to determine the frequency of in utero fetal death (IUFD) related to placental disorders and to assess the frequency of antiphospholipid antibodies syndrome (APS) among women referred to the internal medicine department. METHODOLOGY: A retrospective clinical study conducted in Rennes University Hospital, France. From January 2007 to December 2014, 53 women who presented an IUFD at 14 weeks or more of gestational age were included. The main cause for each IUFD was determined by expert agreement...
July 27, 2017: International Journal of Rheumatic Diseases
Jingran Ma, Hongmei Song, Min Wei, Yanyan He
The study aims to analyse the clinical and immunological manifestations of paediatric antiphospholipid syndrome (APS) in patients, based on the 2006 revised classification criteria of definite APS. Fifty-eight paediatric patients with APS were enrolled and analysed retrospectively. A total of 37 female and 21 male patients with a mean age of 14 ± 3 years at disease onset were included. Fourteen (24%) cases were primary APS, and 40 (69%) cases were secondary to systemic lupus erythaematosus (SLE). Anti-nuclear antibody (ANA) positivity and hypocomplementemia were more common in secondary APS than in primary APS...
July 26, 2017: Clinical Rheumatology
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
Kuo-Tung Tang, Tsu-Yi Hsieh, Ya-Hsuan Chao, Meng-Xian Lin, Yi-Hsing Chen, Der-Yuan Chen, Chi-Chen Lin
INTRODUCTION: Many studies have demonstrated elevated circulating levels of high-mobility group box 1 (HMGB1) and decreased circulating levels of soluble receptor for advanced glycation end products (sRAGE) in patients with autoimmune diseases. In the present study, we investigated plasma levels of both HMGB1 and sRAGE in primary antiphospholipid syndrome (pAPS) patients. METHODS: We prospectively recruited 11 pAPS patients, 17 antiphospholipid antibody (APA)-positive SLE patients without APS manifestations (APA+SLE) and 12 SLE patients with secondary APS (APS+SLE)...
2017: PloS One
Ghulam Murtaza, Joy Iskandar, Tara Humphrey, Sujeen Adhikari, Aneesh Kuruvilla
Libman-Sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves. In most cases, patients do not have significant valvular dysfunction. However, patients with significant valvular dysfunction may present with serious complications such as cardiac failure, arrhythmias, and thromboembolic events. Recently, association of Libman-Sacks endocarditis with antiphospholipid antibody syndrome (APS) has been made. APS is most commonly defined by venous and arterial thrombosis, recurrent pregnancy loss, and thrombocytopenia...
April 2017: Cardiology Research
Andrea Padricelli, Anna Maria Giribono, Doriana Ferrara, Flavia Spalla, Umberto Bracale, Luca Del Guercio, Giuseppe Servillo, Carlo Ruotolo, Umberto Marcello Bracale
The antiphospholipid syndrome (APS), either primary or secondary form, is considered an autoimmune disease with the presence of at least 1 clinical and 1 laboratory abnormalities as defined by the Sydney criteria. Clinical criteria include vascular thrombosis of venous, artery, small vessel in any organ, and recurrent pregnancy pathologies. Mesenteric ischemia is a rare and threatening manifestation of APS. We herein report a case of a 34-year-old pregnant woman referred to our Emergency Room with thoracic and abdominal acute pain...
October 2017: Annals of Vascular Surgery
Emmanouil Papadakis, Anastasia Banti, Anna Kioumi
Antiphospholipid syndrome (APS) is an autoimmune systemic disease characterized by vascular thrombosis (arterial or venous) and/or pregnancy complications associated with the occurrence of autoantibodies, specifically lupus anticoagulant, anticardiolipin antibodies, and/or anti-β2 glycoprotein-I antibodies confirmed at least twice over a 12 week period according to the 2006 Sydney criteria. Antiphospholipid antibodies are encountered  in the general population with a reported prevalence of 1% to 5%  However, APS is far more infrequent with a prevalence of 40-50/100,000 persons and an incidence of about 5 new patients/100,000 persons...
September 2016: Israel Medical Association Journal: IMAJ
Praveen Ramakrishnan Geethakumari, Patrick Mille, Rakesh Gulati, Srikanth Nagalla
Antiphospholipid antibody syndrome (APS) is an enigmatic heterogeneous disorder despite several revelations in its pathobiology. Renal transplantation in patients with APS has been notoriously difficult due to the high risk of development of thrombotic microangiopathy (TMA), which is often refractory to conventional treatment modalities such as aggressive anticoagulation and plasmapheresis. We describe a case of a 58-year-old male with secondary APS undergoing living unrelated renal transplantation for end-stage renal disease from lupus nephritis...
March 10, 2017: Transfusion and Apheresis Science
Carlos A Hinojosa, Rene Lizola, Hugo Laparra-Escareno, Javier E Anaya-Ayala
Inferior vena cava (IVC) filters are useful adjuncts to prevent venous thromboembolism to the pulmonary circulation in the setting of contraindication for anticoagulation. Despite their proven decreased rate of pulmonary embolism, IVC filters are not without complications. We herein present the case of a 22-year-old man with a history of antiphospholipid antibody syndrome who was sent to our institution for evaluation with Budd-Chiari and post-thrombotic syndromes associated to a chronic retrohepatic complete IVC occlusion secondary to an IVC filter placed 5 years earlier...
April 6, 2017: BMJ Case Reports
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