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https://www.readbyqxmd.com/read/28794515/the-complement-system-as-a-potential-therapeutic-target-in-rheumatic-disease
#1
REVIEW
Leendert A Trouw, Matthew C Pickering, Anna M Blom
Complement activation is associated with common rheumatic diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and systemic vasculitis. Evidence linking complement activation to these diseases includes the presence of complement deposition in affected tissues, decreased levels of complement proteins and high levels of complement activation fragments in the blood and/or synovial fluid of patients with these diseases, as well as data from experimental models. Eculizumab, a monoclonal antibody that inhibits the complement component C5, is now approved for the treatment of rare conditions involving complement hyperactivation, and the success of this therapy has renewed interest in understanding the utility of complement inhibition in rheumatological practice, particularly for SLE...
August 10, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28748509/clinical-characteristics-and-thrombosis-outcomes-of-paediatric-antiphospholipid-syndrome-analysis-of-58-patients
#2
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
https://www.readbyqxmd.com/read/28741233/how-to-identify-high-risk-aps-patients-clinical-utility-and-predictive-values-of-validated-scores
#3
REVIEW
Kenji Oku, Olga Amengual, Shinsuke Yasuda, Tatsuya Atsumi
PURPOSE OF REVIEW: Antiphospholipid syndrome (APS) is a clinical disorder characterised by thrombosis and/or pregnancy morbidity in the persistence of antiphospholipid (aPL) antibodies that are pathogenic and have pro-coagulant activities. Thrombosis in APS tends to recur and require prophylaxis; however, the stereotypical treatment for APS patients is inadequate and stratification of the thrombotic risks is important as aPL are prevalently observed in various diseases or elderly population...
August 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28718063/new-insights-on-platelets-and-platelet-derived-microparticles-in-systemic-lupus-erythematosus
#4
REVIEW
Marc Scherlinger, Vanja Sisirak, Christophe Richez, Estibaliz Lazaro, Pierre Duffau, Patrick Blanco
PURPOSE OF REVIEW: Current knowledge on the role of platelets and platelet-derived microparticles (PMPs) on the immune system has been fast-growing. Systemic lupus erythematosus (SLE) is a systemic auto-immune disorder characterized by a loss of tolerance toward nuclear auto-antigens. Although recent studies allowed a better understanding of SLE pathogenesis, there is an urgent need for the development of new treatments and the identification of new biomarkers to assess the disease activity...
August 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28659045/effects-of-statins-on-thrombosis-development-in-patients-with-systemic-lupus-erythematosus-and-antiphospholipid-antibodies
#5
T Watanabe, K Oku, O Amengual, R Hisada, K Ohmura, I Nakagawa, H Shida, T Bohgaki, T Horita, S Yasuda, T Atsumi
The objective of this study is to identify the effects of statins and risk factors for thrombosis in patients with new onset of systemic lupus erythematosus (SLE) with or without antiphospholipid antibodies (aPL). Consecutive patients with SLE without history of thrombotic events were retrospectively enrolled from April 1997 to February 2014. The development of first thrombosis and death caused by thrombosis were defined as the study endpoint. Risk and protective factors for developing thrombosis were analyzed...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#6
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28619369/mechanisms-of-atherosclerosis-and-cardiovascular-disease-in-antiphospholipid-syndrome-and-systemic-lupus-erythematosus-new-therapeutic-approaches
#7
REVIEW
Chary Lopez-Pedrera, M Ángeles Aguirre-Zamorano, Carlos Pérez-Sánchez
Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are 2 highly related autoimmune-rheumatic diseases associated with an increased risk of developing cardiovascular (CV) diseases. Despite the great progresses made in understanding the pathological mechanisms leading to CV diseases in those pathologies, there is still the unmet need to improve long term prognosis. CV diseases in SLE and APS is thought to happen as the result of a complex interaction between traditional CV risk factors, immune deregulation and disease activity, including the synergic effect of cytokines, chemokines, adipokines, proteases, autoantibodies, adhesion receptors, oxidative stress and a plethora of intracellular signalling molecules...
June 12, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28593703/effect-of-ethnicity-on-clinical-presentation-and-risk-of-antiphospholipid-syndrome-in-roma-and-caucasian-patients-with-systemic-lupus-erythematosus-a-multicenter-cross-sectional-study
#8
Victoria Manzano-Gamero, Alfredo J Pardo-Cabello, José A Vargas-Hitos, Mónica Zamora-Pasadas, Nuria Navarrete-Navarrete, José M Sabio, Laura Jáimez-Gámiz, Raquel Ríos-Fernandez, Norberto Ortego-Centeno, M Mar Ayala-Gutierrez, Enrique de Ramón, Agustín Colodro-Ruíz, Luisa Micó-Giner, María J Castillo-Palma, Ángel Robles-Marhuenda, Juan de Dios Luna-Del Castillo, Juan Jiménez-Alonso
AIM: To determine if there are ethnic differences in the prevalence of antiphospholipid syndrome (APS), clinical presentation and autoantibody profile between Roma and Caucasian patients with systemic lupus erythematosus (SLE). METHOD: A cross-sectional study was conducted including data from Roma and Caucasian SLE patients consecutively attending six hospitals in Spain. Socio-demographic characteristics, prevalence of APS, clinical and analytical features of SLE and APS were compared between ethnic groups...
June 7, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28507183/longterm-hydroxychloroquine-therapy-and-low-dose-aspirin-may-have-an-additive-effectiveness-in-the-primary-prevention-of-cardiovascular-events-in-patients-with-systemic-lupus-erythematosus
#9
Serena Fasano, Luciana Pierro, Ilenia Pantano, Michele Iudici, Gabriele Valentini
OBJECTIVE: Systemic lupus erythematosus (SLE) is associated with an increased risk of cardiovascular disease (CVD). Thromboprophylaxis with low-dose aspirin (ASA) and hydroxychloroquine (HCQ) seems promising in SLE. We investigated the effects of HCQ cumulative dosages (c-HCQ) and the possible synergistic efficacy of ASA and HCQ in preventing a first CV event (CVE) in patients with SLE. METHODS: Patients consecutively admitted to our center who, at admission, satisfied the 1997 American College of Rheumatology and/or 2012 Systemic Lupus Collaborating Clinics classification criteria for SLE, and had not experienced any CVE, were enrolled...
July 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28462538/a-comparitive-study-of-anticardiolipin-antibodies-among-systemic-lupus-erythematosus-patients-from-western-and-eastern-india
#10
D Doley, S Kakati, L Saikia, A Rajadhyaksha, Milind Nadkar, P Khadilkar, M Patwardhan, V Pradhan
INTRODUCTION: Anti-phospholipid antibodies (APA) like anticardiolipin antibodies (ACA) are important cause of venous and arterial thrombosis and other occlusive vascular diseases. Prevalence of these antibodies in SLE patients at the time of diagnosis is not known in Indian SLE patients. This study was conducted to evaluate the prevalence of ACA in SLE patients from Eastern and Western India and to correlate them with disease activity. MATERIAL AND METHODS: Seventy SLE patients from Assam Medical College, Dibrugarh, Assam and 85 SLE patients from Rheumatology Department, KEM Hospital, Mumbai were studied...
March 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28401195/live-birth-pregnancy-outcome-after-first-in-vitro-fertilization-treatment-in-a-patient-with-systemic-lupus-erythematosus-and-isolated-high-positive-iga-anti-%C3%AE-2glycoprotein-i-antibodies-a-case-report
#11
Hristina Andreeva, Marit Seip, Stanislava Koycheva
IgA anti-β2glycoprotein I antibodies (IgA-anti-β2GPI) seems to be the most prevalent isotype in patients with Systemic Lupus Erythematosus (SLE) with a significant association to thrombotic events. Both SLE and antiphospholipid syndrome (APS) can be associated with implantation failure, fetal loss and obstetric complications. Recent reports highlight the clinical value of IgA-anti-β2GPI determination in supporting in vitro fertilization (IVF) treatment and IVF pregnancy outcomes. We report a 36-year-old female diagnosed with SLE, endometriosis and unexplained infertility...
January 2017: Open Medicine (Warsaw, Poland)
https://www.readbyqxmd.com/read/28393653/anti-pentraxin-3-auto-antibodies-might-be-protective-in-lupus-nephritis-a-large-cohort-study
#12
Mo Yuan, Ying Tan, Yun Pang, Yong-Zhe Li, Yan Song, Feng Yu, Ming-Hui Zhao
OBJECTIVES: Anti-pentraxin 3 (PTX3) auto-antibodies were found to be associated with the absence of renal involvement in systemic lupus erythematosus (SLE). This study is to investigate the prevalence of anti-PTX3 auto-antibodies and their clinical significance based on a large Chinese lupus nephritis cohort. METHODS: One hundred and ninety-six active lupus nephritis patients, 150 SLE patients without clinical renal involvement, and 100 healthy controls were enrolled...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28363116/clinical-significance-of-anti-domain-1-%C3%AE-2-glycoprotein-i-antibodies-in-antiphospholipid-syndrome
#13
Teresa Iwaniec, Marcin P Kaczor, Magdalena Celińska-Löwenhoff, Stanisław Polański, Jacek Musiał
BACKGROUND: Antiphospholipid syndrome (APS) is characterized by the presence of circulating antiphospholipid antibodies (aPL) in patients with thrombosis and/or pregnancy morbidity. In APS patients anti-domain 1 β2-glycoprotein I (anti-D1 β2GPI) IgG antibodies correlate strongly with thrombosis and to the lesser extent, with pregnancy complications. The aim of this study was to assess clinical utility of the anti-D1 β2GPI antibodies in the diagnosis and risk stratification of antiphospholipid syndrome...
May 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28361572/characterization-of-the-thrombin-generation-profile-in-systemic-lupus-erythematosus
#14
A Kern, E Barabás, A Balog, Sz Burcsár, M Kiszelák, B Vásárhelyi
Systemic lupus erythematosus (SLE) is a multisystemic inflammatory autoimmune disorder. Thrombotic events occur at a higher incidence among SLE patients. The investigation of thrombin generation (TG) with calibrated automated thrombogram (CAT) test as a global hemostasis assay is applicable for the overall functional assessment of the hemostasis. The aim of this study was to characterize the hemostatic alterations observed in SLE by CAT assay. In this study, CAT parameters and basic coagulation parameters of SLE patients (n = 22) and healthy control subjects (n = 34) were compared...
March 1, 2017: Physiology International
https://www.readbyqxmd.com/read/28355983/mesenteric-inflammatory-veno-occlusive-disease-of-the-spleen-metasynchronous-with-two-arterial-thrombotic-events-in-systemic-lupus-erythematosus
#15
D Allali, G Puppa, C Chizzolini
Vasculitides, particularly those affecting small vessels, are known to complicate systemic lupus erythematosus (SLE); however, isolated venulitis of the mesenteric bed has rarely been reported. Here we relate the case of a 46-year-old woman with SLE who presented with acute abdominal pain due to artery thrombosis and extended splenic ischemia requiring splenectomy. The histological examination revealed diffuse venulitis in the absence of arterial vasculitis consistent with the definition of mesenteric inflammatory veno-occlusive disease (MIVOD)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28265683/-recurrent-thromboembolisms-despite-full-anticoagulation-in-a-patient-with-antiphospholipid-syndrome
#16
S Wernicke, K Selleng, S B Felix, A Greinacher, F Hammer
We report on a female patient with confirmed secondary antiphospholipid syndrome (APS) due to underlying systemic lupus erythematosus (SLE). Despite a thromboplastin time within the normal range (international normalized ratio, INR) under treatment with a vitamin K antagonist (VKA), a recurrent thrombotic event occurred, this time as pulmonary embolism due to bilateral deep vein thrombosis. Despite an INR value in the therapeutic range, clotting factors II, VII, IX and X were found to be insufficiently decreased suggesting inefficient anticoagulation...
March 6, 2017: Der Internist
https://www.readbyqxmd.com/read/28258475/expanding-the-therapeutic-options-for-renal-involvement-in-lupus-eculizumab-available-evidence
#17
REVIEW
Savino Sciascia, Massimo Radin, Jinoos Yazdany, Maria Tektonidou, Irene Cecchi, Dario Roccatello, Maria Dall'Era
In this study, we aimed to systematically review available literature on the efficacy of eculizumab for the treatment of renal involvement in patients with systemic lupus erythematosus (SLE). We conducted a literature search developed a priori, to identify articles reporting clinical experience with the use of eculizumab in SLE patients, focusing on renal involvement. The search strategy was applied to Ovid MEDLINE, EMBASE, In-Process and Other Non-Indexed Citation, Cochrane Central Register of Controlled Trials and Scopus from 2006 to present...
August 2017: Rheumatology International
https://www.readbyqxmd.com/read/28130684/clinical-characteristics-and-laboratory-findings-of-252-chinese-patients-with-anti-phospholipid-syndrome-comparison-with-euro-phospholipid-cohort
#18
Hui Shi, Jia-Lin Teng, Yue Sun, Xin-Yao Wu, Qiong-Yi Hu, Hong-Lei Liu, Xiao-Bing Cheng, Yu-Feng Yin, Jun-Na Ye, Pojen P Chen, Cheng-de Yang
This study aims to characterize the Chinese Han patients with anti-phospholipid syndrome (APS) and compare the data with those of the Euro-Phospholipid cohort. We conducted a single center study consisting of 252 patients with definite APS from 2000 to 2015. We analyzed the clinical and laboratory characteristics of our cohort and compared the data with those of the Euro-Phospholipid cohort. Our cohort consisted of 216 females and 36 males, with a mean age at entry into this study of 41 years (range 11-74 years)...
March 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/27989533/the-use-of-direct-oral-anticoagulants-in-56-patients-with-antiphospholipid-syndrome
#19
Katarzyna Malec, Tadeusz Góralczyk, Anetta Undas
INTRODUCTION: Antiphospholipid syndrome (APS) is a common acquired thrombophilia associated with a high thrombotic risk, in which vitamin K antagonists (VKA) represent the mainstay of therapy. Case series involving up to 35 patients with APS suggested limited efficacy and safety of direct oral anticoagulants (DOACs). MATERIAL AND METHODS: In the prospective case series we followed 56 consecutive patients with APS (44 women and 12 men, aged from 22 to 64years), including 33 (60%) associated with systemic lupus erythematosus (SLE) and 16 (28...
April 2017: Thrombosis Research
https://www.readbyqxmd.com/read/27976568/an-unexpected-diagnostic-course-of-systemic-lupus-erythematosus
#20
Aysel Taktak, Adem Köksoy, Özge Başaran, Saba Kiremitçi, Banu Acar, Nermin Uncu, Nilgün Çakar
Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures...
2016: Turkish Journal of Pediatrics
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