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Catastrophic Antiphospholipid Antibody syndrome

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https://www.readbyqxmd.com/read/27886797/catastrophic-antiphospholipid-syndrome-the-current-management-approach
#1
REVIEW
Ignasi Rodriguez-Pintó, Gerard Espinosa, Ricard Cervera
The current recommendation for catastrophic antiphospholipid syndrome (CAPS) management is the standard triple therapy with anticoagulation (AC), glucocorticoids (GCs), plasma exchange (PE), and/or intravenous immunoglobulins (IVIGs). Of note, only AC has a significant effect on the prognosis of these patients. However, from the experimental or basic point of view, there is only indirect evidence to advocate the use of these immunomodulatory therapies (GC, PE, and IVIG) in CAPS. Recently, there have been reports of severe or refractory CAPS patients treated with the monoclonal antibodies rituximab and eculizumab...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27816952/antiphosphatidylserine-prothrombin-antibodies-as-biomarkers-to-identify-severe-primary-antiphospholipid-syndrome
#2
Ariela Hoxha, Elena Mattia, Marta Tonello, Chiara Grava, Vittorio Pengo, Amelia Ruffatti
BACKGROUND: Anti-phosphatidylserine/prothrombin (aPS/PT) antibodies have begun to be considered potentional biomarkers for antiphospholipid syndrome (APS). This cohort study investigate the role of aPS/PT antibodies as a risk factor for severe APS by evaluating the association between those antibodies and clinical/laboratory profiles of APS. METHODS: Plasma/serum samples from 197 APS patients, 100 healthy subjects and 106 patients with autoimmune diseases were collected...
November 7, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/27796482/-therapeutic-administration-of-immunoglobulins
#3
T Witte
BACKGROUND: Intravenously administered immunoglobulins have multiple modes of action that are anti-inflammatory. They can therefore be beneficial in a number of autoimmune disorders. OBJECTIVE: The aim of this article is to analyze and summarize studies on the administration of intravenous immunoglobulins in rheumatological diseases. METHODS: A selective search and analysis of the literature was carried out related to the mode of action and efficacy of intravenous immunoglobulins in rheumatological diseases...
December 2016: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/27745843/catastrophic-antiphospholipid-antibody-syndrome-presenting-as-acute-vascular-occlusion-in-a-young-female-patient
#4
Joaquín Valle Alonso, Francisco Javier Fonseca Del Pozo, Manuel Vaquero Álvarez, Jorge Pedraza, Miguel Angel Aguayo, Almudena Sanchez
Acquired thrombotic and thromboembolic disorders may be presented initially with symptoms and signs of acute ischaemia or organ dysfunction that will lead many of these patients to seek care in the emergency department. We report a case of a 19-year-old female patient who developed catastrophic antiphospholipid syndrome (CAPS syndrome or Asherson syndrome) 6 weeks post stillbirth with an initial presentation of acute vascular occlusion. The patient was immediately operated and anticoagulated with significant improvement...
October 10, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27668204/hepatic-infarction-in-a-pregnant-woman-with-antiphospholipid-syndrome-and-triple-antibody-positivity-a-case-report-focusing-on-catastrophic-antiphospholipid-syndrome
#5
Ji-Hye Kim, Cheonga Yee, Jin-Yi Kuk, Suk-Joo Choi, Soo-Young Oh, Cheong-Rae Roh, Jong-Hwa Kim
Pregnant women with antiphospholipid syndrome (APS) carry a high risk of arterial or venous thrombosis. Such thrombotic conditions occur more frequently in patients with triple positivity to antiphospholipid antibodies or with high antibody titers. Hepatic infarction is a rare complication in pregnant women with APS, and it sometimes mimics HELLP syndrome. This report describes a preeclamptic pregnant woman with APS who had high titers of three antiphospholipid antibodies. She experienced severe epigastric pain with elevated liver enzymes; in addition, she had tachycardia and tachypnea...
September 2016: Obstetrics & Gynecology Science
https://www.readbyqxmd.com/read/27639837/catastrophic-antiphospholipid-syndrome-caps-descriptive-analysis-of-500-patients-from-the-international-caps-registry
#6
Ignasi Rodríguez-Pintó, Marta Moitinho, Irene Santacreu, Yehuda Shoenfeld, Doruk Erkan, Gerard Espinosa, Ricard Cervera
OBJECTIVE: To analyze the clinical and immunologic manifestations of patients with catastrophic antiphospholipid syndrome (CAPS) from the "CAPS Registry". METHODS: The demographic, clinical and serological features of 500 patients included in the website-based "CAPS Registry" were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test while T-test for independent variables was used to compare groups regarding continuous variables...
September 15, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27617783/-2016%C3%A2-review-on-catastrophic-antiphospholipid-syndrome
#7
Nathalie Costedoat-Chalumeau, Laetitia Coutte, Véronique Le Guern, Nathalie Morel, Gaelle Leroux, Romain Paule, Luc Mouthon, Jean-Charles Piette
The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement...
December 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27486689/aps-diagnostics-and-challenges-for-the-future
#8
V Pengo, E Bison, G Zoppellaro, S Padayattil Jose, G Denas, A Hoxha, A Ruffatti, A Banzato
Diagnosis of antiphospholipid syndrome (APS) is essentially based on the detection of circulating antiphospholipid (aPL) antibodies. Progress have been made on the standardization of tests exploring the presence of aPL as guidelines on coagulation and immunological tests were recently published in the literature. Clinical relevance of aPL profile has come from prospective cohort studies in populations with a homogeneous antibody profile supporting the view that triple positivity is a high risk pattern in patients and carriers...
July 31, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27380476/lupus-anticoagulant-hypoprothrombinemia-syndrome-and-catastrophic-antiphospholipid-syndrome-in-a-patient-with-antidomain-i-antibodies
#9
Joris Galland, Shirine Mohamed, Sabine Revuz, Emmanuel de Maistre, Bas de Laat, Pierre-Yves Marie, Stéphane Zuily, Bruno Lévy, Véronique Regnault, Denis Wahl
Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Five years later, he experienced the first systemic lupus erythematous flare...
July 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27158525/placental-histology-and-neutrophil-extracellular-traps-in-lupus-and-pre-eclampsia-pregnancies
#10
Wendy Marder, Jason S Knight, Mariana J Kaplan, Emily C Somers, Xu Zhang, Alexander A O'Dell, Vasantha Padmanabhan, Richard W Lieberman
OBJECTIVE: Systemic lupus erythematosus (SLE) is associated with increased risk of adverse pregnancy outcomes, including pre-eclampsia, particularly in association with antiphospholipid antibody syndrome (APS). While significant placental abnormalities are expected in pre-eclampsia, less is known about how lupus activity and APS in pregnancy affect the placenta. We describe placental pathology from a population of lupus pregnancies, several of which were complicated by APS-related thromboses, in which pre-eclampsia and other complications developed...
2016: Lupus Science & Medicine
https://www.readbyqxmd.com/read/27023104/catastrophic-antiphospholipid-syndrome-in-pregnancy-a-diagnosis-that-should-not-be-missed
#11
Jennifer G Hoayek, Hind N Moussa, Hina A Rehman, Susan Hosseini Nasab, Sean C Blackwell, Baha M Sibai
Catastrophic antiphospholipid syndrome (CAPS) is an accelerated form of the antiphospholipid antibody syndrome resulting in multi-organ ischemia and failure. It is a rare and life-threatening condition that can be easily mistaken with hemolysis elevated liver enzymes low platelets syndrome, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. In order to make a diagnosis, it is required to have multi-organ thrombosis over 1 week affecting at least three organs or systems, and to have positive antiphospholipid antibody on two occasions (6 weeks apart), and histopathologic confirmation of small vessel occlusion...
December 2016: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/27020721/the-role-of-complement-inhibition-in-thrombotic-angiopathies-and-antiphospholipid-syndrome
#12
Doruk Erkan, Jane E Salmon
Antiphospholipid syndrome (APS) is characterized by thrombosis (arterial, venous, small vessel) and/or pregnancy morbidity occurring in patients with persistently positive antiphospholipid antibodies (aPL). Catastrophic APS is the most severe form of the disease, characterized by multiple organ thromboses occurring in a short period and commonly associated with thrombotic microangiopathy (TMA). Similar to patients with complement regulatory gene mutations developing TMA, increased complement activation on endothelial cells plays a role in hypercoagulability in aPL-positive patients...
March 5, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/26927441/treatment-of-catastrophic-antiphospholipid-syndrome
#13
Nayef M Kazzaz, W Joseph McCune, Jason S Knight
PURPOSE OF REVIEW: Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS. RECENT FINDINGS: Data from the international 'CAPS registry', spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and precipitating diagnoses...
May 2016: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/26882790/autosplenectomy-causing-catastrophic-pneumococcal-meningitis-in-a-patient-with-lupus-antiphospholipid-antibody-syndrome
#14
Khushboo Sheth, Aaron Snyder, Ulysses Wu, Bimalin Lahiri, Prashant Grover
We present the case ofa26-year-old female who presented to the hospital with pneumococcal meningitis. A review of her records showed atrophic spleen, and a hypercoagulable workup was positive for Systemic Lupus Erythematous (SLE)/Antiphospholipid Antibody Syndrome (APS). An autosplenectomy from thrombotic occlusion of the splenic artery made her susceptible to pneumococcal meningitis. Autoimmune conditions, particularly SLE and APS, are important causes of hypercoagulable states in a young population, and earlier detection of these conditions and appropriate treatment helps to decrease morbidity and mortality among these patients...
January 2016: Connecticut Medicine
https://www.readbyqxmd.com/read/26329147/two-cases-of-thrombosis-in-patients-with-antiphospholipid-antibodies-during-treatment-of-immune-thrombocytopenia-with-romiplostim-a-thrombopoietin-receptor-agonist
#15
Brian LaMoreaux, Fatima Barbar-Smiley, Stacy Ardoin, Hareth Madhoun
INTRODUCTION: Romiplostim is a thrombopeitin-receptor agonist approved for raising platelet counts in patients with immune thrombocytopenia (ITP). Several hematologic adverse effects have been reported including acute myeloid leukemia, myelofibrosis, and thrombosis. METHODS: We report two cases, one pediatric and one adult patient, who had antiphospholipid antibodies and received romiplostim for ITP. Additionally, we conducted medline, Food and Drug Administration (FDA) Adverse Events reports website, and manufacturer's adverse events database...
February 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/26307097/diagnosis-and-therapy-of-antiphospholipid-syndrome
#16
Vittorio Pengo, Gentian Denas, Seena J Padayattil, Giacomo Zoppellaro, Elisa Bison, Alessandra Banzato, Ariela Hoxha, Amelia Ruffatti
Antiphospholipid syndrome (APS) is a clinical condition that has not been well defined yet. Although the clinical component is well established, the laboratory part is a mood issue. According to current guidelines, 3 tests (lupus anticoagulant, anticardiolipin, and anti β2-glycoprotein I antibodies) are officially recommended to assess the presence of antiphospholipid antibodies. According to test positivity, patients are classified into categories in clinical studies. However, it is now clear that classification categories have a different impact on the clinical course of APS...
2015: Polskie Archiwum Medycyny Wewnętrznej
https://www.readbyqxmd.com/read/26209907/the-role-of-infectious-diseases-in-the-catastrophic-antiphospholipid-syndrome
#17
REVIEW
M Garcia-Carrasco, C Mendoza-Pinto, S Macias-Diaz, F Vazquez de Lara, I Etchegaray-Morales, J L Galvez-Romero, S Mendez-Martinez, R Cervera
Catastrophic antiphospholipid syndrome (CAPS), also called "Asherson syndrome", is a variant of the antiphospholipid syndrome (APS) that occurs in less than 1% of APS cases. The etiology of CAPS is uncertain; however, several triggering factors have been recognized. The most common of these are infectious diseases, particularly those of the respiratory tract. CAPS pathogenesis is incompletely understood, but several theories have been proposed, such as the molecular mimicry theory, which describes the production of anti-β2-glycoprotein I (GP1) antibody in response to infection...
November 2015: Autoimmunity Reviews
https://www.readbyqxmd.com/read/26157953/crohn-s-disease-causes-a-catastrophe
#18
Anam Khan, Yamini Natarajan, Joseph Sellin
Patients with Crohn's disease are at higher risk of developing antiphospholipid antibody syndrome (APS), of which 1% develop life-threatening, rapidly progressive clotting known as catastrophic APS (CAPS). A 17-year-old male presented with intermittent bloody diarrhea and abdominal pain. He developed myopericarditis, alveolar hemorrhage, left frontal and parieto-occipital infarct, superior sagittal sinus venous thrombosis, disseminated intravascular coagulation, and a pulmonary embolus. He was treated with high-dose pulse steroids, anticoagulation, and plasma exchange...
April 2015: ACG Case Reports Journal
https://www.readbyqxmd.com/read/26014099/a-case-of-catastrophic-antiphospholipid-syndrome-first-report-with-advanced-cardiac-imaging-using-mri
#19
A N Rosenbaum, N S Anavekar, F C Ernste, S V Mankad, R J Le, K K Manocha, G W Barsness
This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS)...
October 2015: Lupus
https://www.readbyqxmd.com/read/26000150/treatment-of-the-antiphospholipid-syndrome
#20
REVIEW
Monica Galli
The antiphospholipid syndrome is characterized by a combination of laboratory findings (i.e., the presence of at least one antiphospholipid antibody) and clinical manifestations (arterial and/or venous thrombosis, obstetrical complications). Long-term oral anticoagulant is recommended to prevent recurrence of both arterial and venous thrombosis, whereas (low molecular weight) heparin plus aspirin is the treatment of choice to prevent further obstetrical complications. In the rare case of catastrophic antiphospholipid syndrome, heparin plus high-dose corticosteroids plus plasma exchange is associated with the highest recovery rate...
June 2014: Auto- Immunity Highlights
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