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1.
Rev Med Suisse ; 16(689): 670-674, 2020 Apr 08.
Artigo em Francês | MEDLINE | ID: mdl-32270932

RESUMO

The antiphospholipid syndrome (APS) is a complex autoimmune -disease characterized by the expression of antiphospholipid -antibodies (APL) and a variety of clinical presentation. The latest classification defines APS by the occurrence of vascular thrombosis and/or typical obstetrical morbidity together with persistently -detectable APL at least 12 weeks apart. The latest recommendation proposes a risk profile based on the type and titer of APL detected, in order to guide the intensity of prophylactic measures. Based on current knowledge, novel oral anticoagulants should not be used in APS, particularly in patients with a high-risk APL profile or arterial thrombosis. Beyond the mere aspect of anticoagulant treatment, immunomodulatory approaches to the APS such as hydroxychloroquine are under investigation.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica , Trombose , Adulto , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Conhecimento , Gravidez , Complicações na Gravidez/sangue , Complicações na Gravidez/tratamento farmacológico , Fatores de Risco , Trombose/sangue , Trombose/tratamento farmacológico , Trombose/etiologia
3.
S D Med ; 72(10): 464-466, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31816208

RESUMO

Catastrophic antiphospholipid syndrome (CAPS) is a rare disorder characterized by acute, multi-system dysfunc- tion due to small-vessel thrombosis related to anti-phospholipid antibodies. Here we present an unusual case of CAPS presenting with genitourinary manifestations. A 72-year-old male developed a series of symptoms over the course of two weeks. His symptoms included testicular inflammation, scrotal edema, priapism, hematuria, penile eschar, elbow eschars, and acute kidney injury. He was found to have anti-phospholipid antibodies and treated with anticoagulation, high-dose steroids and plasma exchange. His symptoms resolved with minimal lasting effects. This case is unique to the literature because of the extensive genitourinary involvement.


Assuntos
Síndrome Antifosfolipídica , Trombose , Idoso , Anticorpos Antinucleares/sangue , Anticorpos Antifosfolipídeos , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Glucocorticoides/uso terapêutico , Humanos , Masculino , Troca Plasmática/métodos , Plasmaferese , Trombose/etiologia , Trombose/patologia
4.
Rinsho Shinkeigaku ; 59(10): 662-665, 2019 Oct 26.
Artigo em Japonês | MEDLINE | ID: mdl-31564704

RESUMO

A 55-year-old man presented with recurrent brain infarction which had increased multifocally mainly in the cerebral white matter over the course of one year. Antibodies associated with antiphospholipid syndrome (APS) were initially negative. The patient was admitted to our department because of the thickened meninges shown on gadolinium enhanced brain MRI, mimicking hypertrophic pachymeningitis. However, blood and cerebrospinal fluid analysis revealed no significant inflammatory changes. On histopathological examination of the biopsied meninges, the arachnoid membrane was thickened with fibrosis, and arachnoidal microvessels were enlarged without significant inflammatory changes. The dura mater was not thickened, and no inflammation or microvessel enlargement were revealed. Finally, serum IgG anticardiolipin antibody testing was positive twice at an interval of more than 12 weeks, confirming the diagnosis of APS. Since initiating antithrombotic therapy with warfarin, brain infarction has not recurred. Without inflammation in the arachnoid membrane, the congestion of blood flow caused by thrombosis of microvessels in the arachnoid membrane might have increased the thickness of the arachnoid membrane.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Aracnoide-Máter/patologia , Infarto Cerebral/etiologia , Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/tratamento farmacológico , Aracnoide-Máter/irrigação sanguínea , Aracnoide-Máter/diagnóstico por imagem , Biomarcadores/sangue , Cardiolipinas/imunologia , Infarto Cerebral/prevenção & controle , Humanos , Hipertrofia/etiologia , Imunoglobulina G/sangue , Imagem por Ressonância Magnética , Masculino , Microvasos , Pessoa de Meia-Idade , Recidiva , Trombose/complicações , Resultado do Tratamento , Varfarina/administração & dosagem
5.
Autoimmun Rev ; 18(12): 102408, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31648041

RESUMO

Secondary thromboprophylaxis with low molecular heparin or vitamin K antagonists (VKAs) is recommended in patients with definite antiphospholipid syndrome (APS). Direct oral anticoagulant (DOACs) have been approved in different prothrombotic conditions and have numerous advantages compared to VKAs. Whether DOACs can be used for secondary prophylaxis in APS is an open question. Data from the TRAPS randomized controlled Trial, meta-analysis and case reports indicate that we should not treat patients with triple positive APS and/or arterial thrombi with routine doses of DOACS. On the other hand, data from the literature including, case series, meta- analysis and the RAPS trial indicate that there are low risk patients, such as patients who suffered from a venous but not an arterial thromboembolism and are LAC negative who may benefit from the treatment with DOACs. Prospective trials addressing these low risk patients are needed in order to consider DOAC treatment in such patients.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Humanos
6.
Vasa ; 48(6): 483-486, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31621546

RESUMO

The antiphospholipid-syndrome (APS) is one of the most severe forms of thrombophilia, which may not only lead to recurrent venous but also to arterial thromboembolic events (TE), and to severe pregnancy complications, respectively. APS is defined by clinical symptoms and specific laboratory findings: 1. Lupus anticoagulant (LA), 2. anticardiolipin-antibodies (ACA), and 3. ß2-Glycoprotein I-antibodies (ß2GPI-Ab). All test results have to be confirmed after at least 12 weeks. The thrombotic risk is highest, if all 3 test groups are positive. It must be pointed out that the presence of UFH, VKA or DOACs may lead to false positive LA-test results; the addition of a specific absorber after blood sampling may provide reliable results in the presence of DOACs. A prospective randomized controlled trial comparing warfarin and rivaroxaban (TRAPS-trial) including only high-risk patients with triple positive APS was terminated early because of an increased rate of TE in patients treated with rivaroxaban [19 %, mostly arterial, compared to 3 % with warfarin (HR 7.4;1.7-32.9)]. Subsequently, a warning letter was issued by the pharmaceutical manufacturers of DOACs, including a warning of DOAC use in APS-patients, particularly in triple-positive high-risk patients. Conclusions: 1. Clinical suspicion of APS requires careful diagnostic testing. Because of inadequate diagnostic workup, many patients may not even have an APS, and these patients could be adequately treated with a DOAC. 2. Patients with single or double positive antiphospholipid antibodies but without positive LA may have a comparably low thrombotic risk and may also be treated with a DOAC in venous TE - sufficient evidence for that conclusion is not yet available but is suggested by the results of meta-analyses. 3. Triple positive patients or those with APS who suffered from arterial thromboembolism have a very high recurrence risk of thrombosis; the TRAPS-Study shows that these patients should be treated with VKA instead of a DOAC.


Assuntos
Síndrome Antifosfolipídica , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Humanos , Estudos Prospectivos , Rivaroxabana/uso terapêutico , Varfarina/uso terapêutico
7.
BMJ Case Rep ; 12(9)2019 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-31527209

RESUMO

Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially life-threatening variant of the antiphospholipid syndrome which is characterised by multiple small vessel thrombosis which can lead to multiorgan failure. CAPS is a clinical emergency which all clinicians need to be aware of because early diagnosis and treatment may improve maternal and fetal outcome. Here, we report a case of CAPS in pregnancy in a 31-year-old female patient who presented at 28 weeks of gestation. A literature review of CAPS in pregnancy and the puerperium is also included.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Glucocorticoides/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Complicações na Gravidez/tratamento farmacológico , Adulto , Síndrome Antifosfolipídica/diagnóstico por imagem , Doença Catastrófica , Cesárea , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Humanos , Gravidez , Complicações na Gravidez/diagnóstico por imagem , Resultado da Gravidez , Adulto Jovem
8.
Blood Coagul Fibrinolysis ; 30(7): 364-365, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31464688

RESUMO

: Vitamin K antagonists (VKA) remain the treatment of choice for catastrophic antiphosphilipid syndrome (CAPS). However, when VKAs do not work for a specific patient, direct oral anticoagulants (DOAC) may be a valid therapeutic alternative. We present a patient with a psychiatric disorder and CAPS who was noncompliant to VKA and low-molecular-weight heparin. He was started on dabigatran and has remained thrombosis-free for 8 years. Due to CAPS he has developed progressive renal failure but dabigatran levels were within the expected range. In conclusion, this case report provides anecdotic evidence that dabigatran may be of use in patients with high-risk APS in whom VKA are not an option.


Assuntos
Síndrome Antifosfolipídica/tratamento farmacológico , Dabigatrana/uso terapêutico , Anticoagulantes/uso terapêutico , Doença Catastrófica , Inibidores do Fator Xa/uso terapêutico , Humanos , Masculino , Resultado do Tratamento , Vitamina K/antagonistas & inibidores
9.
J Am Podiatr Med Assoc ; 109(3): 235-240, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31268794

RESUMO

Antiphospholipid syndrome is an autoimmune disease characterized by vascular thrombosis involving both the arterial and venous systems that can lead to tissue ischemia or end-organ damage. Much of the literature describes various symptoms at initial presentation, but isolated tissue ischemia manifesting as a solitary blue toe is unusual. We discuss a case of a 23-year-old man who presented to the emergency department with a solitary blue fourth digit with minimal erythema and edema, who was suffering from exquisite pain. Following an extensive workup, the patient was diagnosed with antiphospholipid syndrome with thrombi of the vasculature in their lower extremity. With therapeutic anticoagulation, the patient's symptoms subsided and amputation of the digit was prevented.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/diagnóstico , Síndrome do Artelho Azul/etiologia , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Angiografia por Tomografia Computadorizada , Diagnóstico Diferencial , Enoxaparina/uso terapêutico , Pé/irrigação sanguínea , Pé/diagnóstico por imagem , Humanos , Masculino , Dor/etiologia , Artérias da Tíbia/diagnóstico por imagem , Dedos do Pé/irrigação sanguínea , Varfarina/uso terapêutico , Adulto Jovem
10.
Clin Exp Med ; 19(3): 281-288, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31214910

RESUMO

Catastrophic antiphospholipid syndrome (CAPS) is a rare disorder, characterized by the development of multiple vascular thrombosis over a short period of time, in patients with persistently detectable antiphospholipid antibodies (aPLs). The vascular occlusions predominantly affect small vessels. The overall mortality is 36.9%, despite the recent progress in the therapeutic approach. It has been shown that aPLs are able to induce a hypercoagulability state through different mechanisms of action, including complement activation, which in turn plays a key role in the pathogenesis of some thrombotic microangiopathies. Consequently, complement inhibition may be proposed as a targeted intervention to effectively prevent the progression of the microthrombotic storm. The employment of the complement inhibitor eculizumab has been proposed in CAPS on the basis of occasional reports and expert opinion. We report the case of a 54-year-old woman with a CAPS refractory to conventional therapies, who was successfully treated with eculizumab. The administration of this anti-C5 monoclonal antibody aborted the acute progressive thrombotic events and prevented further clinical episodes of thrombosis in the following year. We also faced our case to a systematic literature review, by analyzing all reported cases of CAPS in which eculizumab was added to conventional therapy. Even if further investigation is needed, our results suggest that the inhibition of one mechanism of aPL-induced organ damage may be an add-on treatment for this condition.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/patologia , Fatores Imunológicos/uso terapêutico , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
13.
Lupus ; 28(8): 1003-1006, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31122135

RESUMO

OBJECTIVES: We discuss two patients with antiphospholipid syndrome (APS) who presented with critical ischemia of both lower extremities due to arterial microthrombi. They received multimodality therapy emergently: anticoagulation, immunosuppression, and therapeutic plasma exchange (TPE). Then they were maintained on anticoagulation with fondaparinux and immunosuppression with mycophenolate mofetil (MMF), and were followed for 4 years. METHODS: Two patients with APS with ischemia and necrosis of their distal lower extremities were treated emergently with anticoagulation (intravenous heparin), immunosuppression (prednisone), and TPE. They were maintained on anticoagulation with fondaparinux and immunosuppression with MMF. RESULTS: Neither patient had recurrent microthrombotic disease during a 4-year follow-up. CONCLUSIONS: As described in our small cohort, patients with APS who suffer from microthrombotic arterial disease may benefit from maintenance therapy of anticoagulation with fondaparinux and immunosuppression with MMF, an approach which may be worthy of further trial. Fondaparinux does not require attention to diet, monitoring, and cumbersome bridging that is typical of warfarin therapy. MMF provides immunosuppression while sparing the side effects of steroid treatment.


Assuntos
Síndrome Antifosfolipídica/tratamento farmacológico , Fondaparinux/uso terapêutico , Ácido Micofenólico/uso terapêutico , Adulto , Anticoagulantes/uso terapêutico , Terapia Combinada , Feminino , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Troca Plasmática , Trombose/etiologia , Trombose/prevenção & controle , Resultado do Tratamento
14.
BMJ Case Rep ; 12(4)2019 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-30940667

RESUMO

A 63-year-old woman with known antiphospholipid syndrome (APLS) presented with catastrophic APLS and multiorgan dysfunction after a change in her anticoagulation from warfarin to rivaroxaban. Evidence suggests direct-acting oral anticoagulants (DOACs) like rivaroxaban may be less effective than warfarin in secondary prevention of thrombotic events in high-risk APLS patients.


Assuntos
Síndrome Antifosfolipídica/tratamento farmacológico , Rivaroxabana/administração & dosagem , Prevenção Secundária , Trombose/prevenção & controle , Administração Oral , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/complicações , Inibidores do Fator Xa/administração & dosagem , Inibidores do Fator Xa/efeitos adversos , Feminino , Humanos , Pessoa de Meia-Idade , Rivaroxabana/efeitos adversos , Trombose/etiologia , Varfarina/uso terapêutico
16.
Thromb Haemost ; 119(7): 1147-1153, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31018220

RESUMO

Antiphospholipid antibodies (aPL) have been reported to activate platelets. This is considered to be one of the pathogenic properties of aPL. Even though aPL heterogeneity is quite well established, little is known, if the ability to activate platelets is common to all aPL or depends on antigen specificity. To further study this issue, we analyzed the ability of three human monoclonal aPL with distinctly different antigenic specificities to activate platelets in vitro. The results obtained with human monoclonal aPL were validated with immunoglobulin G (IgG) fractions obtained from patients with antiphospholipid syndrome (APS). A co-factor-independent human monoclonal anticardiolipin aPL had no discernible effect on human platelets. Two monoclonal aPL reactive against ß2 glycoprotein I (ß2GPI) induced platelet aggregation, integrin αIIbß3 activation and P-selectin surface expression. These data could be confirmed with patient IgG fractions which could only induce aggregation, if they had anti-ß2GPI activity. Anti-ß2GPI aPL-induced platelet activation depended on interaction of aPL with the low affinity Fcγ-receptor IIa on the platelet surface. It was completely abolished by pretreatment of platelet-rich plasma with the mechanistic target of rapamycin (mTOR) inhibitors rapamycin or everolimus. This extends previous data showing that mTOR is involved in signaling of anti-ß2GPI in monocytes and endothelial cells. In conclusion, anti-ß2GPI aPL activate platelets while co-factor-independent anticardiolipin aPL have no effect. mTOR is involved in this signaling process which has implications beyond APS, because so far the role of mTOR signaling in platelets is incompletely explored and requires further study.


Assuntos
Síndrome Antifosfolipídica/imunologia , Plaquetas/fisiologia , Epitopos de Linfócito B/metabolismo , Epitopos/metabolismo , Agregação Plaquetária/efeitos dos fármacos , Anticorpos Antifosfolipídeos/metabolismo , Síndrome Antifosfolipídica/tratamento farmacológico , Autoanticorpos/metabolismo , Células Cultivadas , Everolimo/farmacologia , Everolimo/uso terapêutico , Humanos , Complexo Glicoproteico GPIIb-IIIa de Plaquetas/metabolismo , Transdução de Sinais , Sirolimo/farmacologia , Sirolimo/uso terapêutico , Serina-Treonina Quinases TOR/antagonistas & inibidores , beta 2-Glicoproteína I/imunologia
17.
Nat Commun ; 10(1): 1916, 2019 04 23.
Artigo em Inglês | MEDLINE | ID: mdl-31015489

RESUMO

Potentiation of neutrophil extracellular trap (NET) release is one mechanism by which antiphospholipid antibodies (aPL Abs) effect thrombotic events in patients with antiphospholipid syndrome (APS). Surface adenosine receptors trigger cyclic AMP (cAMP) formation in neutrophils, and this mechanism has been proposed to regulate NETosis in some contexts. Here we report that selective agonism of the adenosine A2A receptor (CGS21680) suppresses aPL Ab-mediated NETosis in protein kinase A-dependent fashion. CGS21680 also reduces thrombosis in the inferior vena cavae of both control mice and mice administered aPL Abs. The antithrombotic medication dipyridamole is known to potentiate adenosine signaling by increasing extracellular concentrations of adenosine and interfering with the breakdown of cAMP. Like CGS21680, dipyridamole suppresses aPL Ab-mediated NETosis via the adenosine A2A receptor and mitigates venous thrombosis in mice. In summary, these data suggest an anti-inflammatory therapeutic paradigm in APS, which may extend to thrombotic disease in the general population.


Assuntos
Agonistas do Receptor A2 de Adenosina/farmacologia , Adenosina/análogos & derivados , Síndrome Antifosfolipídica/tratamento farmacológico , Armadilhas Extracelulares/efeitos dos fármacos , Neutrófilos/efeitos dos fármacos , Fenetilaminas/farmacologia , Trombose Venosa/tratamento farmacológico , Adenosina/imunologia , Adenosina/metabolismo , Adenosina/farmacologia , Animais , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/genética , Síndrome Antifosfolipídica/imunologia , Síndrome Antifosfolipídica/patologia , AMP Cíclico/imunologia , AMP Cíclico/metabolismo , Dipiridamol/farmacologia , Modelos Animais de Doenças , Armadilhas Extracelulares/imunologia , Armadilhas Extracelulares/metabolismo , Fibrinolíticos/farmacologia , Regulação da Expressão Gênica , Humanos , Imunoglobulina G/sangue , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Neutrófilos/imunologia , Receptor A2A de Adenosina/genética , Receptor A2A de Adenosina/imunologia , Transdução de Sinais , Veia Cava Inferior/efeitos dos fármacos , Veia Cava Inferior/imunologia , Veia Cava Inferior/metabolismo , Trombose Venosa/genética , Trombose Venosa/imunologia , Trombose Venosa/patologia
18.
Medicine (Baltimore) ; 98(8): e14619, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30813192

RESUMO

BACKGROUND: Previous clinical studies reported low molecular weight heparin (LMWH) monotherpay has been utilized for the treatment of recurrent abortion (RCA) with antiphospholipid system (APS). However, its efficacy is still inconclusive. This systematic review aims to assess its efficacy and safety for patients with RCA and APS. METHODS: A systematic literature search for article up to February 2019 will be conducted in 9 databases: Cochrane Library, EMBASE, MEDILINE, Cumulative Index to Nursing and Allied Health Literature, Allied and Complementary Medicine Database, Chinese Biomedical Literature Database, China National Knowledge Infrastructure, VIP Information, and Wanfang Data. Inclusion criteria are randomized control trials of LMWH monotherpay for patients with RCA and APS. The Cochrane risk of bias tool will be used to evaluate the methodological quality for each qualified study. The summary results will be showed by using fixed-effects and random-effects models for pooling the data based on the heterogeneity of included studies. RESULTS: This systematic review will assess the clinical efficacy and safety of LMWH monotherpay in treating RCA with APS. The primary outcome is pregnancy loss. The secondary outcomes include frequency of preterm delivery, live birth rates, maternal and fetal complications, as well as adverse events. CONCLUSION: The findings of this study will summarize the present evidence to judge whether LMWH monotherpay is an effective therapy for patients with RCA and APS. DISSEMINATION AND ETHICS: The findings of this study will be published by through peer-reviewed journals. This study does not needs ethic documents, because it will not analyze individual patient data. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42019121064.


Assuntos
Aborto Habitual/tratamento farmacológico , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Heparina de Baixo Peso Molecular/uso terapêutico , Aborto Habitual/etiologia , Anticoagulantes/efeitos adversos , Síndrome Antifosfolipídica/complicações , Feminino , Heparina de Baixo Peso Molecular/efeitos adversos , Humanos , Gravidez , Resultado da Gravidez/epidemiologia , Projetos de Pesquisa , Resultado do Tratamento
19.
PLoS One ; 14(3): e0212614, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30870459

RESUMO

Antiphospholipid antibodies (aPL) promote endothelial dysfunction, inflammation and procoagulant state. We investigated the effect of hydroxychloroquine (HCQ) on prothrombotic state and endothelial function in mice and in human aortic endothelial cells (HAEC). Human aPL were injected to C57BL/6 mice treated or not with HCQ. Vascular endothelial function and eNOS were assessed in isolated mesenteric arteries. Thrombosis was assessed both in vitro by measuring thrombin generation time (TGT) and tissue factor (TF) expression and in vivo by the measurement of the time to occlusion in carotid and the total thrombosis area in mesenteric arteries. TGT, TF, and VCAM1 expression were evaluated in HAEC. aPL increased VCAM-1 expression and reduced endothelium dependent relaxation to acetylcholine. In parallel, aPL shortened the time to occlusion and extended thrombus area in mice. This was associated with an overexpression of TF and an increased TGT in mice and in HAEC. HCQ reduced clot formation as well as TGT, and improved endothelial-dependent relaxations. Finally, HCQ increased the p-eNOS/eNOS ratio. This study provides new evidence that HCQ improves procoagulant status and vascular function in APS by modulating eNOS, leading to an improvement in the production of NO.


Assuntos
Síndrome Antifosfolipídica/tratamento farmacológico , Hidroxicloroquina/farmacologia , Trombose/prevenção & controle , Animais , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/imunologia , Síndrome Antifosfolipídica/patologia , Modelos Animais de Doenças , Células Endoteliais/imunologia , Células Endoteliais/patologia , Endotélio Vascular/imunologia , Endotélio Vascular/patologia , Feminino , Humanos , Masculino , Camundongos , Óxido Nítrico Sintase Tipo III/imunologia , Trombina/imunologia , Tromboplastina/imunologia , Trombose/imunologia , Trombose/patologia , Molécula 1 de Adesão de Célula Vascular/imunologia
20.
BMJ Case Rep ; 12(3)2019 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-30898956

RESUMO

Catastrophic antiphospholipid syndrome (CAPS) is a rare but severe form of antiphospholipid syndrome (APS). The syndrome manifests itself as a rapidly progressive multiorgan failure that is believed to be caused by widespread micro-thrombosis. Seldom does bleeding comanifest with thrombosis. We present a patient with APS who presented with nausea, vomiting and fatigue, and rapidly progressed into multiorgan failure before being diagnosed with CAPS. The clinical course was complicated by an atraumatic intracranial haemorrhage which demanded discontinuation of anticoagulation. The patient was treated with high dose steroid, intravenous immunoglobulin, followed by weekly rituximab infusion. Although the trigger for CAPS was not obvious during her hospital stay, she was diagnosed with acute cytomegalovirus (CMV) infection soon after discharge. In this case report, we explore the differential diagnoses of CAPS, investigate the possibility of CMV infection as a potential trigger, present the therapeutic challenges of anticoagulation and discuss the emerging use of rituximab.


Assuntos
Síndrome Antifosfolipídica/complicações , Hemorragias Intracranianas/etiologia , Adulto , Síndrome Antifosfolipídica/tratamento farmacológico , Doença Catastrófica , Diagnóstico Diferencial , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Hemorragias Intracranianas/diagnóstico por imagem , Imagem por Ressonância Magnética , Rituximab/administração & dosagem
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