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Antiphospolipid syndrome

Jordana Vaz Hendler, Lucian de Souza, Daniele Corrêa de Freitas Zernow, Juliano Guimarães, Thiago Bertotto, Carla Forgiarini Saldanha, João Carlos Tavares Brenol, Odirlei André Monticielo
Systemic lupus erythematosus (SLE) treatments progress over the years. However, the mortality remains higher than in the general population. Few studies have examined SLE patients' survival in Brazil. This study aims to identify the main characteristics and risk factors to predict mortality and recognize the main causes of death in Brazilian patients with SLE. We retrospectively assessed clinical, demographic, and serological characteristics from 600 patients followed since 2001 in SLE outpatient clinic from Hospital de Clínicas de Porto Alegre...
September 2017: Clinical Rheumatology
Francisco Cardoso
Chorea, a movement disorder characterised by a continuous flow of unpredictable muscle contractions, has a myriad of genetic and non-genetic causes. Although autoimmune processes are rare aetiology of chorea, they are relevant both for researchers and clinicians. The aim of this article is to provide a review of the epidemiology, clinical and laboratory features, pathogenesis and management of the most common autoimmune causes of chorea. Emphasis is given particularly to Sydenham's chorea, systemic lupus erythematosus, primary antiphospolipid antibody syndrome, paraneoplastic chorea and anti- N -methyl-d-aspartate receptor encephalitis...
May 2017: Journal of Neurology, Neurosurgery, and Psychiatry
Sophie Legrève
OBJECTIVE: Multifocal osteonecrosis is rare. A zone of epiphyseal bone-marrow necrosis of three or more joints characterizes it. The most common risk factors are glucocorticoids intake and alcoholism. However, there is a series of other causes that could be incriminated in osteonecrosis, among which antiphospholipid syndrome. This case report, illustrates a primary antiphospholipid syndrome with only one clinical manifestation: multifocal osteonecrosis. OBSERVATIONS: A 30-year-old woman, with a medical history of left total hip replacement (THR), presents with severe right coxalgia and pain in both knees and shoulders...
September 2016: Annals of Physical and Rehabilitation Medicine
Ignasi Rodríguez-Pintó, Ricard Cervera, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is characterized by thrombosis in more than three organs or systems developing over a short period of time. Despite conventional treatment with a combination of anticoagulation plus corticosteroids plus plasma exchange, and/or intravenous immunoglobulin, mortality remains high and some patients suffer from recurrent CAPS episodes. In selected patients, new therapies such as rituximab may be a treatment option. In this review, the rationale for using rituximab in CAPS is discussed...
February 2015: Therapeutic Advances in Musculoskeletal Disease
Tuba Kuz Tekşut, Halil Özcan, Mein Işık, Fatih Karslı
Chorea gravidarum (CG) is a rare movement disorder characterized by rapid, irregular randomly distributed involuntary movements during pregnancy. Similar to Sydenham chorea, psychiatric symptoms may be observed in cases of CG. CG may be idiopathic or secondary to an underlying cause. One of the most common causes of CG is antiphospholipid syndrome. Herein we present a case of recurrent CG that was considered to be due to antiphospholipid syndrome. The patient had a history of 3 pregnancy losses and her fourth pregnancy was treated appropriately, resulting in the birth of healthy full-term baby...
2013: Türk Psikiyatri Dergisi, Turkish Journal of Psychiatry
Carlos A Núñez-Álvarez, Javier Cabiedes
The antiphospolipid syndrome (APS) is an autoimmune disease characterized by recurrent fetal loss, thrombotic events (arterial or venous) and hemocytopenic disorders associated to high titers of circulating aPL. Two variants of the APS have been described. Primary APS is a clinical entity without evidence of any other autoimmune disease and secondary APS is a clinical disorder mainly associated with Systemic Lupus Erithematosus (SLE). aPL are a widely group of immunoglobulins directed against different components or proteins factors...
January 2011: Reumatología Clinica
Hiroshi Ogishima, Satoshi Ito, Akito Tsutsumi, Makoto Sugihara, Daisuke Goto, Isao Matsumoto, Mana Obata-Yasuoka, Hiromi Hamada, Hiroyuki Yoshikawa, Hideto Takahashi, Atsuko Murashima, Takayuki Sumida
A case of a 37-year-old pregnant patient with antiphospholipid syndrome (APS), who has a medical history of both thrombosis and recurrent fetal loss, is presented. She was treated with predonisolone and fixed-dose unfractionated heparin (UFH) infusion, followed by plasmaphereses and fixed-dose low-molecular-weight heparin infusion during her fourth pregnancy. Unfortunately, this treatment did not have beneficial effects, resulting in intrauterine growth restriction and finally neonatal death. Continuous intravenous UFH infusion and low-dose aspirin were administrated under the monitoring of the activated partial thromboplastin time to achieve a target level of 120 s during her fifth pregnancy...
August 2010: International Journal of Rheumatic Diseases
B Obón Azuara, M R Ortas Nadal, I Gutiérrez Cía, R Bustamante Rodríguez, C Velilla Soriano, B Villanueva Anadón
Catastrophic antiphospolipid syndrome (CAPS) is extremely rare antiphospolipid syndrome (APS) variety associated to higher mortality. When heart involvement appears has worsening pronostic. We reported a CAPS case, possibily afterward sting wasp triggering, with acute heart failure during evolution. The patient died despite angiographic stents, anticoagulation, corticoids and plasmaphereses treatment carried out.
May 2008: Anales de Medicina Interna: Organo Oficial de la Sociedad Española de Medicina Interna
Rolf T Urbanus, Ronald H M W Derksen, Philip G de Groot
The diagnosis of the antiphospholipid syndrome, a non-inflammatory autoimmune disease characterized by thrombosis or pregnancy morbidity in the presence of antiphospholipid antibodies, depends greatly upon laboratory diagnostics. The diagnostic value of all available assays to detect antiphospholipid antibodies and the anticardiolipin assay in particular, is a matter of ongoing debate. Although the presence of lupus anticoagulant correlates best with thrombosis, accurate determination is not always possible due to anticoagulant treatment...
March 2008: Blood Reviews
R Manna, V Ricci, V Curigliano, M Pomponi, F Adamo, A Costa, G De Socio, G Garbarrini, F D'Onofrio, P Bria
Antiphospholipid syndrome is a disorder characterised by recurrent venous or arterial thrombosis and/or foetal losses associated with typical laboratory abnormalities. The initial manifestation of anthiphospholipid syndrome can involve many organ systems either singly or in combination. We describe the case of a 62 yr old female showing schizophrenia-like symptoms in which further evaluations allowed us to diagnose the antiphospolipid syndrome.
October 2006: International Journal of Immunopathology and Pharmacology
Dino Veneri, Achille Ambrosetti, Massimo Franchini, Federico Mosna, Giovanni Poli, Giovanni Pizzolo
The association of lymphoid neoplasms and antiphospolipid antibodies (APA), with or without thromboembolic complications, has been reported in several cases. We describe one case of B-cell non-Hodgkinís lymphoma (NHL) in which the combination of rituximab with standard chemotherapy led to the complete remission of a severe hypercoagulable state associated with APA.
November 2005: Haematologica
Dennis A Nowak, Sebastian Mutzenbach, Hans H Fuchs
Forty nine consecutive cases of acute myelopathy were related to the following pathologies: 31 MS, four spinal cord infarction, four parainfectious, one antiphospolipid syndrome and nine of unknown origin. Sensory deficits were most frequent in MS and in myelopathy of unknown origin. In spinal cord infarction motor deficits and sphincter dysfunction were present in all cases. In parainfectious myelopathy sensory-motor deficits and sphincter dysfunction were most frequent. Myelopathy extended over less than two vertebral segments in MS and in myelopathy of unknown origin...
February 2004: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
N L Kozlovskaia, M M Ibragimova, P E Krel
No abstract text is available yet for this article.
2000: Klinicheskaia Meditsina
P Dürig, C Ferrier, H Schneider
Hypertension in pregnancy is defined by a systolic blood pressure > or = 140 mm Hg and a diastolic blood pressure of > or = 90 mm Hg or by a rise in blood pressure of systolic > or = 30 mm Hg and diastolic > or = 15 mm Hg. High blood pressures are found in 5-10% of all pregnancies. The outcome of pregnancy is influenced by the fact whether there occurs a proteinuria in addition to hypertension. While the prognosis of an isolated hypotension is good, the combination of hypertension and proteinuria leading to preeclampsia is the primary cause of maternal death in many countries and is responsible for 20-25% of perinatal mortality...
October 1999: Therapeutische Umschau. Revue Thérapeutique
T Barbui, G Finazzi, M Galli
Antiphospholipid antibodies are a wide ranging, heterogeneous family of autoantibodies, formerly believed to be directed to anionic phospholipids. Recent research, however, has confirmed that they are directed to plasma proteins bound to suitable (phospholipid) anionic surfaces. The most well-known and best characterized antigens are beta 2-glycoprotein I, recognized by anticardiolipin antibodies, and prothrombin, recognized by most lupus anticoagulants. Lupus anticoagulants are generally identified on the basis of their capacity to prolong the phospholipid-dependent coagulation tests...
April 1997: Annali Italiani di Medicina Interna: Organo Ufficiale Della Società Italiana di Medicina Interna
S E Ford, L Kennedy, P M Ford
The clinicopathologic correlations of antiphospholipid antibodies (aPLs) have so far only been examined in case reports and highly selected series. This study assessed the incidence of aPLs in 156 consecutive, unselected autopsies and correlated the pathological findings with the clinical histories. Elevations of aPLs were found in 20.5% of the autopsy population, compared with 9.6% of age- and sex-matched controls and 2% of healthy normal subjects. There was a higher incidence of thromboembolic disease in patients with elevated aPL levels compared with those without, but the histology of thrombi was similar in both groups, with no evidence of vasculitis in the aPL-positive individuals...
May 1994: Archives of Pathology & Laboratory Medicine
H Boudoulas, M Vavuranakis, C F Wooley
The many changes in classification of cardiovascular disease during the twentieth century reflect changing etiology of diseases, clinical comprehension and technological advances. In particular, the etiology of valvular heart disease has changed dramatically in the last five decades. The significant reduction of acute rheumatic fever and its sequelae, and the recognition of non-rheumatic causes of valvular disease are responsible for the metamorphosis in the etiology of valvular disorders. Valvular heart disease can be classified as follows: 1) Heritable-congenital causes of valvular heart disease e...
September 1994: Journal of Heart Valve Disease
S Bulvik, I Aronson, S Ress, P Jacobs
Bone marrow necrosis (BMN), defined morphologically by destruction of hematopoietic tissue, including the stroma, with preservation of the bone, is a rare syndrome. The conditions in which it is seen include sickle cell disease, acute leukemia, metastatic neoplasia, and bacterial infection, particularly when hypovolemia and septic shock are present. BMN is also associated with disseminated intravascular coagulation (DIC) following irradiation and antineoplastic therapy. The antiphospolipid syndrome (APS) is characterized by antibodies directed against the antiphospolipid substrate...
June 1995: American Journal of Medicine
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