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https://www.readbyqxmd.com/read/29320972/rituximab-use-in-pediatric-lupus-anticoagulant-hypoprothrombinemia-syndrome-report-of-three-cases-and-review-of-the-literature
#1
K Cetin Gedik, S Siddique, C L Aguiar
Lupus anticoagulant hypoprothrombinemia syndrome (LA-HPS) is a rare condition that may predispose both to thrombosis and bleeding due to positive lupus anticoagulant (LA) and factor II (FII) deficiency. It can be seen in association with infections or systemic lupus erythematosus (SLE) and may require glucocorticoids (GCs) and/or immunosuppressive medications. Pediatric LA-HPS cases in the literature and three cases that received only rituximab (RTX) for LA-HPS (in addition to GCs) at two institutions between January 2010 and June 2017 were analyzed descriptively...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29301604/bilateral-choroidal-occlusion-in-antiphospholipid-syndrome-associated-with-systemic-lupus-erythematosus
#2
Yang Zhang, Shun-Hua Zhang, Ai-Ling Bian, You-Xin Chen
This article reports a rare case of bilateral choroidal occlusion that occurred in a 24-year-old woman with antiphospholipid syndrome (APS) associated with systemic lupus erythematosus (SLE). This young lady concurred with aorta ventralis thrombosis and bilateral iliac artery occlusion when presented, and experienced a rapid deterioration of vision. She also has a history of recurrent miscarriage. Corticosteroid, immunosuppression and anticoagulation therapy were administered. Patients with APS associated with SLE are at risk for thrombotic phenomena, which may affect the ocular vessels of all sizes, including choroidal vessel...
December 30, 2017: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/29301470/risk-of-complications-of-ultrasound-guided-renal-biopsy-for-adult-and-pediatric-patients-with-systemic-lupus-erythematosus
#3
Y S Sun, I T Sun, H K Wang, A H Yang, C Y Tsai, C J Huang, D F Huang, C C Lai
Objective The objective of this paper is to identify the risk of complications of real-time ultrasound-guided renal biopsy in adult and pediatric patients with systemic lupus erythematosus (SLE). Materials and methods This retrospective study examined outcomes of 296 renal biopsy procedures in 275 SLE patients. Imaging-confirmed symptomatic hematoma was regarded as a major complication when intervention (blood transfusion, angiographic embolization, or surgery) was required or as a minor complication otherwise...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29295895/impaired-contraction-of-blood-clots-as-a-novel-prothrombotic-mechanism-in-systemic-lupus-erythematosus
#4
Giang Le Minh, Alina D Peshkova, Izabella A Andrianova, Timur B Sibgatullin, Adelia N Maksudova, John W Weisel, Rustem Litvinov
The aim of this work was to examine a possible role of clot contraction/retraction in thrombotic complications of systemic lupus erythematosus (SLE). Using a novel automated method, we investigated kinetics of clot contraction in the blood of 51 SLE patients and 60 healthy donors. The functionality of platelets in the SLE patients was assessed using flow cytometry by expression of P-selectin and fibrinogen-binding capacity. The rate and degree of clot contraction were significantly reduced in SLE patients compared to healthy subjects, especially in the patients with higher blood levels of anti-dsDNA antibodies...
January 2, 2018: Clinical Science (1979-)
https://www.readbyqxmd.com/read/29166262/antiphosphatidylserine-prothrombin-antibodies-aps-pt-as-potential-diagnostic-markers-and-risk-predictors-of-venous-thrombosis-and-obstetric-complications-in-antiphospholipid-syndrome
#5
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
https://www.readbyqxmd.com/read/29150207/carotid-and-femoral-atherosclerosis-in-antiphospholipid-syndrome-equivalent-risk-with-diabetes-mellitus-in-a-case-control-study
#6
Evrydiki Kravvariti, George Konstantonis, Nikolas Tentolouris, Petros P Sfikakis, Maria G Tektonidou
BACKGROUND: Antiphospholipid syndrome (APS) may carry a worse prognosis for vascular complications when co-existing with subclinical atherosclerosis; however, the association between the two conditions remains ambiguous. METHODS: We evaluated ultrasonographic markers of subclinical atherosclerosis in carotid and femoral arteries of 86 patients with thrombotic APS [43 primary APS (PAPS), 43 systemic lupus erythematosus-associated APS (SLE/APS)], 86 patients with diabetes mellitus (DM) and 86 healthy controls, individually matched for age and gender, and investigated their associations with traditional and disease-related factors in APS...
October 23, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29050535/antiphospholipid-antibodies-and-non-thrombotic-manifestations-of-systemic-lupus-erythematosus
#7
U İlgen, M E Yayla, A Ateş, İ E Okatan, E U Yurteri, M Torgutalp, A B D Keleşoğlu, T M Turgay, G Kınıklı
Objectives The aim of this study was to investigate the association between antiphospholipid antibodies and non-thrombotic and non-gestational manifestations of systemic lupus erythematosus. Methods Systemic lupus erythematosus patients with persistently positive antiphospholipid antibodies or lupus anticoagulant were identified and grouped as systemic lupus erythematosus with antiphospholipid syndrome (SLE-APS), systemic lupus erythematosus with positive antiphospholipid antibodies/lupus anticoagulant without antiphospholipid syndrome (SLE-aPL), and systemic lupus erythematosus with negative aPLs (SLE-No aPL)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28976051/coagulopathy-as-a-complication-of-kidney-biopsies-in-paediatric-systemic-lupus-erythematosus-patients-with-antiphospholipid-syndrome
#8
Hiroko Nagata, Mai Sato, Masao Ogura, Takahisa Yoshikawa, Kazuna Yamamoto, Sohshi Matsumura, Yuji Kano, Ken Saida, Mayumi Sako, Koichi Kamei, Takako Yoshioka, Kentaro Ogata, Shuichi Ito, Kenji Ishikura
Children with systemic lupus erythematosus (SLE) generally undergo a pretreatment kidney biopsy. However, some of these patients, especially those with antiphospholipid syndrome (APS), may experience serious coagulopathic complications. We report herein two cases of paediatric SLE with APS in which, despite normal blood test results, the disparate coagulopathic complications of haemorrhage and embolism developed following a kidney biopsy. Case 1 was, an 8-year-old male in whom, primary APS was initially diagnosed...
October 4, 2017: Nephrology
https://www.readbyqxmd.com/read/28933234/risk-of-damage-and-mortality-in-sle-patients-fulfilling-the-acr-or-only-the-slicc-classification-criteria-a-10-year-inception-cohort-study
#9
L Inês, M Rodrigues, D Jesus, F P Fonseca, J A P Silva
Objective To compare damage and mortality, from inception up to 10-year follow-up, between SLE patients meeting at baseline the 1997 ACR criteria or only the 2012 SLICC classification criteria. Methods Patients fulfilling the ACR and/or the SLICC classification criteria for SLE were enrolled at inception and followed-up to 10 years at an academic lupus clinic. Damage was defined as SLICC Damage Index (SDI) score ≥1. We assessed with multivariate Cox models the damage and mortality outcomes, according to SLE classification status at inception, adjusting for potential baseline confounders...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28905254/use-of-eculizumab-in-a-systemic-lupus-erythemathosus-patient-presenting-thrombotic-microangiopathy-and-heterozygous-deletion-in-cfhr1-cfhr3-a-case-report-and-systematic-review
#10
REVIEW
Maria Izabel de Holanda, Luis Cristóvão Pôrto, Teresa Wagner, Luis Fernando Christiani, Lilian M P Palma
The association of thrombotic microangiopathy (TMA) with systemic lupus erythematosus (SLE) has been described in 0.5 to 10% of cases, and patients present worse outcome. TMA is described as the association of microangiopathic hemolytic anemia, thrombocytopenia, and an organ injury, frequently the kidney. This study describes a successful case of use of eculizumab in a patient with SLE and TMA refractory to standard therapy, and provides a literature review. Case description and search in PubMed and MEDLINE using systemic lupus erythemathous and/or antiphospholipid syndrome (APS) and eculizumab retrieved 15 case reports...
December 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28856466/tma-secondary-to-sle-rituximab-improves-overall-but-not-renal-survival
#11
Fangfang Sun, Xiaodong Wang, Wanlong Wu, Kaiwen Wang, Zhiwei Chen, Ting Li, Shuang Ye
Thrombotic microangiopathy (TMA) includes a series of life-threatening disorders. Systemic lupus erythematosus (SLE) is one of the most common acquired causes. To identify predictors of prognosis in patients with TMA secondary to SLE, we conducted a single-center historical study. From January 2013 to June 2016, of 2182 SLE hospitalized patients in the Ren Ji Hospital, a total of 21 consecutive patients with TMA secondary to SLE were identified. The 90-day short-term mortality was 33.3%. The kidney involvement (66...
August 30, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28794515/the-complement-system-as-a-potential-therapeutic-target-in-rheumatic-disease
#12
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...
September 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28748509/clinical-characteristics-and-thrombosis-outcomes-of-paediatric-antiphospholipid-syndrome-analysis-of-58-patients
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
August 22, 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
#19
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
#20
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
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