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https://www.readbyqxmd.com/read/27913748/a-3-year-follow-up-of-a-patient-with-acute-renal-failure-caused-by-thrombotic-microangiopathy-related-to-antiphospholipid-syndrome-case-report
#1
X-J Zhou, M Chen, S-X Wang, F-D Zhou, M-H Zhao
BACKGROUND: Microvascular manifestations of antiphospholipid antibody syndrome in the kidneys include acute renal failure, thrombotic microangiopathy and hypertension. Therapy has been largely empiric. CASE REPORT: A 49-year-old Chinese man presented with anuric acute renal failure without abundant proteinuria and heavy haematuria, but markedly low levels of urinary sodium, potassium and chlorine upon admission. On day 1 of hospitalization, his thrombocytopenia, anaemia and renal failure showed rapid progression...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27913565/a-24-year-old-male-with-a-painful-and-cold-lower-extremity
#2
Eric A Robinson, Zain I Khalpey, Rajesh Janardhanan
CLINICAL INTRODUCTION: A 24-year-old male presented to the emergency department with intense pain in his right lower extremity. He has a medical history significant for systemic lupus erythematosus and antiphospholipid syndrome. He also had four prior episodes of deep venous thromboses on rivaroxaban. The patient stated that early in the morning, he started to feel intense pain that started from his knee and progressed to his calf, with associated numbness and paraesthesia. On physical examination, the limb felt cold with absent right popliteal and dorsalis pedis pulses...
December 2, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27913551/what-is-the-role-of-hydroxychloroquine-in-reducing-thrombotic-risk-in-patients-with-antiphospholipid-antibodies
#3
Tzu-Fei Wang, Wendy Lim
A 35-year-old man presents with an acute unprovoked deep vein thrombosis of the left lower extremity. He is treated with anticoagulation and elects to discontinue treatment after 6 months. He subsequently develops polyarthralgias, fatigue, and a malar rash, and a diagnosis of systemic lupus erythematosus is made based on laboratory and clinical findings. Additional laboratory testing reveals persistent triple positive antiphospholipid antibodies, including lupus anticoagulant, high titer anticardiolipin antibodies, and anti-β2-glycoprotein I antibodies...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27903029/-lupus-erythematosus-update-2016
#4
Martin Aringer, Reinhard Edmund Voll
Meanwhile, five years have passed since the approval of the anti-BAFF antibody belimumab as a first biological for SLE, but no further SLE drug candidate is even close to approval. There are still no clinical trial data available for the use of new oral anticoagulants in antiphospholipid syndrome. In spite of convincing evidence for the use of mycophenolate mofetil (MMF) in lupus nephritis, the German "Gemeinsame Bundesausschuss" (GBA) has not yet decided on its reimbursement. However, several of the ongoing clinical trials have potential to lead to important advances in SLE treatment in the future...
November 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27881058/relationship-between-systemic-lupus-erythematosus-activity-and-persistent-positive-antiphospholipid-antibodies
#5
Zhaleh Shariati Sarabi, Maryam Sahebari, Ali Etemad Rezaie, Mohammadtaghi Norouzi, Kamila Hashemzadeh
Systemic Lupus Erythematosus (SLE) is an autoimmune disorder, characterized by producing different auto-antibodies and multiorgan involvements. In this study we aimed to investigate the relationship between SLE activity and persistently positive antiphospholipid antibodies. Fifty-nine lupus patients (55 women and 4 men) who were assessed in two consecutive visits with 6 weeks interval were selected. Patients` clinical and laboratory data and serum antiphospholipid antibodies` values, were collected. Serum anticardiolipin antibodies and lupus anticoagulant were measured in two visits...
November 22, 2016: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/27866289/echocardiographic-findings-in-asymptomatic-systemic-lupus-erythematosus-patients
#6
Abdel GaffarA Mohammed, Abdulaziz A Alghamdi, Mohammad A ALjahlan, Ibrahim A Al-Homood
The aim of this study is to use transthoracic echocardiographic (TTE) imaging methods to identify cardiac dysfunction in asymptomatic systemic lupus erythematosus (SLE) patients and to determine the association between echocardiographic findings and serology. This is a prospective cross-sectional study where 50 patients with confirmed diagnoses of SLE were recruited from rheumatology outpatient clinics. Clinical and serological evaluation to confirm the diagnosis of lupus was done in all patients. Fifty SLE patients, 46 (92%) females and 4 (8%) males, were recruited...
November 19, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27862892/lupus-anticoagulant-hypoprothrombinemia-syndrome-associated-with-severe-thrombocytopenia-in-a-child
#7
Aimee Foord, Nicole Baca, David Buchbinder, Arash Mahajerin
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) comprises lupus anticoagulant, acquired hypoprothrombinemia, and often mild thrombocytopenia or normal platelets. It is usually associated with autoimmunity or postviral illness. We describe a case of a 10-year-old boy with oral bleeding and severe thrombocytopenia initially suggestive of immune thrombocytopenia. Secondary to bleeding, evaluation demonstrated prolonged coagulation tests and subsequently revealed the presence of lupus anticoagulant and hypoprothrombinemia, along with marked autoimmunity, suggestive of LAHPS...
November 15, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27861383/living-donor-renal-transplantation-in-patients-with-antiphospholipid-syndrome-a-case-report
#8
Ji Yoon Choi, Joo Hee Jung, Sung Shin, Young Hoon Kim, Duck Jong Han
INTRODUCTION: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. CLINICAL FINDINGS: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27860336/monitoring-patients-with-the-lupus-anticoagulant-while-treated-with-vitamin-k-antagonists-communication-from-the-ssc-of-the-isth
#9
A Tripodi, B de Laat, D Wahl, W Ageno, B Cosmi, M Crowther
No abstract text is available yet for this article.
November 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27854095/cerebral-venous-sinus-thrombosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome-with-atypical-antibodies-a-case-report
#10
Tsung-Han Ho, Yu-Wei Hsu, Chih-Wei Wang, Jiunn-Tay Lee, Chi-Hsin Ting, Fu-Chi Yang
BACKGROUND: Although Sjögren's syndrome has been known to complicate with white matter lesions, encephalopathy, or stroke, reports of cerebral venous sinus thrombosis due to Sjögren's syndrome with atypical antibodies are rare. CASE REPORT: A 50-year-old woman was admitted to our neurological ward with nausea and vomiting following acute onset of severe headache in the left occipital region. Brain computed tomography revealed no abnormalities. The patient was fully conscious, with normal cognitive functioning, but exhibited unsteady tandem gait...
June 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/27799439/systemic-lupus-erythematosus-in-spanish-males-a-study-of-the-spanish-rheumatology-society-lupus-registry-relesser-cohort
#11
A Riveros Frutos, I Casas, I Rúa-Figueroa, F J López-Longo, J Calvo-Alén, M Galindo, A Fernández-Nebro, J M Pego-Reigosa, A Olivé Marqués
OBJECTIVE: The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. METHODS: A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). RESULTS: Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women)...
October 31, 2016: Lupus
https://www.readbyqxmd.com/read/27776934/the-antiphospholipid-syndrome-in-patients-with-systemic-lupus-erythematosus
#12
Guillermo J Pons-Estel, Laura Andreoli, Francesco Scanzi, Ricard Cervera, Angela Tincani
The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence or absence of SLE might modify the clinical or serological expression of APS. Apart from the classical manifestations, APS patients with associated SLE more frequently display a clinical profile with arthralgias, arthritis, autoimmune hemolytic anemia, livedo reticularis, epilepsy, glomerular thrombosis, and myocardial infarction...
October 21, 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27770664/clinical-implications-of-the-detection-of-antibodies-directed-against-domain-1-of-%C3%AE-2-glycoprotein-1-in-thrombotic-antiphospholipid-syndrome
#13
Silmara Montalvão, Priscila Soares Elídio, Sabrina da Silva Saraiva, Bruna de Moraes Mazetto, Marina Pereira Colella, Erich Vinícius de Paula, Simone Appenzeller, Joyce Annichino-Bizzacchi, Fernanda Andrade Orsi
INTRODUCTION: Antibodies directed against domain 1 of β2 glycoprotein 1 (aβ2GP1-Dm1) have been involved in the immunopathogenesis of antiphospholipid syndrome (APS). However, the clinical relevance of aβ2GP1-Dm1 in thrombotic APS has not yet been fully explored. OBJECTIVES: To determine the frequency of aβ2GP1-Dm1 in a cohort of patients with thrombotic APS, and to evaluate whether testing for aβ2GP1-Dm1 could have a clinical impact upon the risk assessment of the disease...
October 4, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27759698/occlusive-nonvasculitic-vasculopathy-a-review
#14
Mar Llamas-Velasco, Victoria Alegría, Ángel Santos-Briz, Lorenzo Cerroni, Heinz Kutzner, Luis Requena
We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Different disorders may produce nonvasculitic occlusive vasculopathy in cutaneous blood and lymphatic vessels, including embolization due to cholesterol and oxalate emboli, cutaneous intravascular metastasis from visceral malignancies, atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis, intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material...
October 18, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27758970/wide-discrepancies-in-activated-clotting-times-measured-by-two-devices-were-detected-for-a-patient-with-lupus-anticoagulant-in-a-cardiac-surgery
#15
Keika Miyazawa, Yuji Otsuka, Yoshiki Ishiguro
INTRODUCTION: For a patient with lupus anticoagulant (LA), activated coagulation time (ACT) was measured with two different types of devices (HEMOCHRON(®) 801 and HEMOCHRON(®) Jr). CASE REPORT: ACTs during heparinization measured with the HEMOCHRON(®) 801 were over the range of measurement, while those with the HEMOCHRON(®) Jr. reflected an almost normal response to heparin. DISCUSSION: The phospholipid contained in an activating agent of the HEMOCHRON(®) Jr was suggested to have counteracted the effect of LA...
October 5, 2016: Perfusion
https://www.readbyqxmd.com/read/27743753/lupus-anticoagulant-hypoprothrombinemia-syndrome-presenting-with-co-existing-cerebral-venous-thrombosis-and-subdural-hemorrhage
#16
N Bel Feki, S Zayet, I Ben Ghorbel, M-H Houman
The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count...
October 12, 2016: Journal des Maladies Vasculaires
https://www.readbyqxmd.com/read/27730530/a-combined-approach-using-global-coagulation-assays-quickly-differentiates-coagulation-disorders-with-prolonged-aptt-and-low-levels-of-fviii-activity
#17
Tomoko Matsumoto, Keiji Nogami, Midori Shima
Patients with mild/moderate hemophilia (H)A, acquired HA (AHA) and lupus anticoagulants (LA), have prolonged aPTTs with low levels of factor (F)VIII activity, but the differentiation of these disorders is complex and time consuming. We established an approach to quickly differentiate these disorders using comprehensive coagulation tests. Patients' plasmas with mild/moderate HA, AHA, LA without anti-phospholipid syndrome [LA-APS(-)], and LA with APS [LA-APS(+)] were examined using clot waveform analysis (CWA) and thrombin generation test (TGT)...
October 11, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27708976/the-clinical-significance-of-antiphospholipid-antibodies-in-systemic-lupus-erythematosus
#18
Ozan Ünlü, Stephane Zuily, Doruk Erkan
Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage...
June 2016: Eur J Rheumatol
https://www.readbyqxmd.com/read/27704162/tnf-alpha-and-annexin-a2-inflammation-in-thrombotic-primary-antiphospholipid-syndrome
#19
Mirjana Bećarević
Antiphospholipid syndrome (APS) is characterized by thromboses and/or pregnancy losses. Laboratory criterion for the diagnosis of APS is the presence of antiphospholipid antibodies (anticardiolipin, anti-beta2-glycoprotein I (aβ2gpI) and lupus anticoagulant). On the one hand, the latest classification criteria for the diagnosis of APS emphasized that thrombotic manifestations of the syndrome should be without any signs of an inflammatory process, while on the other hand, some recent reports have suggested that APS is a "pro-inflammatory state...
October 4, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27698076/stillbirth-the-impact-of-antiphospholipid-syndrome
#20
C A Herrera, C C Heuser, D Ware Branch
Fetal death resulting in stillbirth is generally acknowledged as a feature of antiphospholipid syndrome. Recently published studies appear to confirm the association between antiphospholipid antibodies (aPL) and stillbirth, though additional studies of better design would be welcome. Emerging evidence suggests that treatment with heparin agents and low dose aspirin to prevent fetal death is imperfect. New therapeutic approaches for patients with lupus anticoagulant or triple aPL positivity are needed.
October 3, 2016: Lupus
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