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1.
Rheumatology (Oxford) ; 59(7): 1489-1494, 2020 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-32359070

RESUMO

The antiphospholipid antibody syndrome (APS), a chronic autoimmune thrombophilia with an increased mortality and morbidity, has been recognized for more than three decades. Unlike other autoimmune rheumatic conditions such as systemic lupus erythematosus, myositis and Sjögren's syndrome, relatively few attempts have been made to develop activity, damage or disease-specific quality of life indices for APS. In this review of the literature, we consider those attempts that have been made to develop assessment tools for patients with APS, but also reflect upon the nature of the condition, to discuss, in particular, whether an activity index is appropriate for this disease.


Assuntos
Síndrome Antifosfolipídica/fisiopatologia , Qualidade de Vida , Tromboembolia/fisiopatologia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/psicologia , Humanos , Avaliação de Resultados em Cuidados de Saúde , Tromboembolia/etiologia
2.
Hamostaseologie ; 40(2): 174-183, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32455458

RESUMO

Despite a lot of research on antiphospholipid antibodies (aPL), standardization of test systems, and better definition of its clinical symptoms, the pathomechanism of this acquired autoimmune disease is not yet fully explained. Progress in treatment increased the live birth rate in 70 to 80% of women suffering from obstetric antiphospholipid syndrome (OAPS). However, still 20 to 30% will develop adverse pregnancy outcome. Lack of awareness of this disorder as the cause for pregnancy complications is very harmful to mothers and to their newborns. Complications can be avoided or minimized by proper treatment. The aim of this article is to increase the awareness of gynecologists and medical personal for OAPS.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Complicações na Gravidez/prevenção & controle , Síndrome Antifosfolipídica/fisiopatologia , Feminino , Humanos , Gravidez
3.
Intern Med ; 59(11): 1457-1460, 2020 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-32161219

RESUMO

We herein report a 26-year-old woman with sudden cardiac arrest who had no remarkable medical history. While resuscitation was successfully performed with adrenalin administration and extracorporeal membrane oxygenation, the cause of cardiac arrest could not be determined for over two weeks. Given the presence of autoimmune disease along with the findings of refractory renal insufficiency and thrombocytopenia, a kidney biopsy and blood examinations, including lupus anticoagulant testing, were performed, which proved the presence of antiphospholipid syndrome. The patient was successfully treated with steroid pulse therapy. This drastic case scenario highlighted the fact that autoimmune disease can be the cause of sudden cardiac arrest.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/fisiopatologia , Morte Súbita Cardíaca/etiologia , Oxigenação por Membrana Extracorpórea/métodos , Pulsoterapia/métodos , Adulto , Feminino , Humanos , Resultado do Tratamento
4.
Lupus ; 29(2): 157-164, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31948351

RESUMO

INTRODUCTION: Antiphospholipid syndrome (APS) is associated with greater atherothrombotic risk and endothelial dysfunction, suggesting that endothelial glycocalyx is impaired in this disease. OBJECTIVES: The aim was to investigate the endothelial glycocalyx and the relationship between glycocalyx markers, endothelial dysfunction parameters and atherosclerotic markers in APS. METHODS: A total of 15 primary arterial APS patients and healthy controls were included in the study. Glycocalyx was assessed in both groups by sublingual sidestream dark field imaging and syndecan-1 plasma level. Endothelial function was evaluated by brachial artery flow-mediated dilatation (FMD) and early atherosclerosis by carotid intima media thickness (IMT). Thrombotic profile was also performed by measuring the plasma level of the tissue factor (TF). RESULTS: APS patients had significantly increased syndecan-1 plasma level 38.6 ± 5.0 pg/ml vs. 19.1 ± 3.5 pg/ml; p < 0.01 and a reduced glycocalyx thickness 0.26 ± 0.03 µm vs. 0.75 ± 0.07 µm; p < 0.01 compared with control. FMD was impaired in APS patients compared with control, 5.68% ± 0.42 vs. 8.29 ± 0.30, p < 0.01, respectively. IMT was significantly increased in APS patients compared with control, 0.52 ± 0.13 mm vs. 0.40 ± 0.06 mm, p < 0.01, respectively. Soluble TF, thiobarbituric acid-reactive substances levels were increased in the sera from APS patients compared with control. CONCLUSIONS: This preliminary study supports, for the first time, that in APS patients endothelial glycocalyx is impaired, which could lead to thrombosis, endothelial dysfunction and early atherosclerosis.


Assuntos
Síndrome Antifosfolipídica/fisiopatologia , Aterosclerose/etiologia , Autoanticorpos/imunologia , Endotélio Vascular/fisiopatologia , Glicocálix/patologia , Trombose/etiologia , Adolescente , Adulto , Idoso , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Biomarcadores/sangue , Artéria Braquial/diagnóstico por imagem , Artérias Carótidas/diagnóstico por imagem , Espessura Intima-Media Carotídea , Estudos de Casos e Controles , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Sindecana-1/sangue , Tromboplastina/análise , Vasodilatação , Adulto Jovem
5.
Artigo em Inglês | MEDLINE | ID: mdl-31679995

RESUMO

Appropriate contraception and preconception counseling are critical for women of reproductive age with systemic autoimmune diseases (AIDs) because clinical diagnosis, rheumatology medications, and disease activity may impact the safety or efficacy of certain contraceptives as well as the risk of adverse pregnancy outcomes. The presence of antiphospholipid (aPL) antibodies (anticardiolipin, anti-ß2 glycoprotein I, and lupus anticoagulant) is the most important determinant of contraception choice, as women with these antibodies should not receive estrogen-containing contraceptives because of the increased risk of thrombosis. Prepregnancy counseling generally includes the assessment of preexisting disease-related organ damage, current disease activity, aPL antibodies, anti-Ro/SS-A and anti-La/SS-B antibodies, and medication safety in pregnancy. Quiescent AID for six months on pregnancy-compatible medications optimizes maternal and fetal/neonatal outcomes for most patients.


Assuntos
Síndrome Antifosfolipídica/fisiopatologia , Doenças Autoimunes/fisiopatologia , Anticoncepção/métodos , Aconselhamento , Cuidado Pré-Concepcional , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/complicações , Doenças Autoimunes/complicações , Feminino , Humanos , Gravidez , Resultado da Gravidez , Fatores de Risco
6.
Lupus ; 28(13): 1503-1509, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31623520

RESUMO

Prognosis of pregnancies in women with antiphospholipid syndrome has dramatically improved over the past two decades using conventional treatment with low molecular weight heparin and low-dose aspirin. However, despite this regimen, 10-15% of antiphospholipid syndrome patients experience pregnancy losses. Several studies have been performed in order to identify risk factors predictive of complications. Thrombosis has been generally accepted as the key pathogenetic mechanism underlying pregnancy morbidity. However, the thrombogenic state alone is not able to explain all the different mechanisms leading to pregnancy failure. In fact, emerging evidence shows that complement pathway could play an important role in mediating clinical events in antiphospholipid syndrome. However, the exact mechanism through which complement mediates antiphospholipid syndrome complications remains unknown. Low complement levels (C3 and C4) are associated with poor pregnancy outcome in women with antiphospholipid syndrome in different studies. Hypocomplementemia could be indicated as an early predictor of adverse pregnancy outcome, available at the beginning of pregnancy for starting, if necessary, additional treatment to conventional therapy. However, future studies need to better understand the impact of low complement level on antiphospholipid syndrome pregnancy outcome.


Assuntos
Síndrome Antifosfolipídica/fisiopatologia , Complicações na Gravidez/fisiopatologia , Resultado da Gravidez , Anticoagulantes/administração & dosagem , Ativação do Complemento , Complemento C3/metabolismo , Complemento C4/metabolismo , Feminino , Humanos , Gravidez , Complicações na Gravidez/sangue , Complicações na Gravidez/imunologia , Prognóstico , Fatores de Risco , Trombose/tratamento farmacológico , Trombose/etiologia
7.
Lupus ; 28(13): 1558-1565, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31635555

RESUMO

OBJECTIVES: The objective of this study was to investigate risk factors of microvascular involvement and survival in Chinese patients with primary antiphospholipid syndrome. METHODS: In this single-center, retrospective study, we enrolled 112 patients with a confirmed diagnosis of primary antiphospholipid syndrome who were admitted to Peking Union Medical College Hospital from January 2004 to December 2016. Demographic data, clinical characteristics, laboratory results, and follow-up records were collected. RESULTS: A total of 112 patients with primary antiphospholipid syndrome were studied. Microvascular involvement was identified in 21 patients (18.75%). Patients with microvascular involvement experienced fewer episodes of arterial or venous thrombosis (28.6% vs. 84.6%) and a higher incidence of thrombocytopenia (85.7% vs. 54.9%), respectively. Low complement and elevated high-sensitivity CRP levels were observed more frequently in the microvascular group compared with the non-microvascular group (complement 38.1% vs. 18.7%; high-sensitivity CRP 71.4% vs. 31.9%, respectively). Anti-ß2-glycoprotein I antibodies were more prevalent in patients with microvascular involvement than in patients without (66.7% vs. 33.0%, respectively). Multivariate logistic regression analysis revealed that thrombocytopenia (odds ratio = 4.523, 95% confidence interval 1.139-17.962), elevated high-sensitivity CRP levels (odds ratio = 6.385, 95% confidence interval 1.969-20.704), and anti-ß2-glycoprotein I antibody positivity (odds ratio = 5.042, 95% confidence interval 1.555-16.352) were independent risk factors for microvascular involvement. A Kaplan-Meier analysis revealed that survival was significantly poorer in patients with microvascular involvement compared with patients without (p = 0.0278). CONCLUSIONS: In addition to arterial and venous thrombosis, antiphospholipid syndrome can affect the microvasculature of select organs. It is thus important for clinicians to be aware that antiphospholipid syndrome-associated microvascular involvement has a unique pathogenesis and can be a life-threatening condition.


Assuntos
Síndrome Antifosfolipídica/fisiopatologia , Trombocitopenia/epidemiologia , Trombose Venosa/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/mortalidade , Proteína C-Reativa/metabolismo , China , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Microvasos/patologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Trombocitopenia/etiologia , Trombose Venosa/etiologia , Adulto Jovem
8.
Rev Bras Ginecol Obstet ; 41(10): 621-627, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31658490

RESUMO

Antiphospholipid antibody syndrome (APS) is a systemic, autoimmune, prothrombotic disease characterized by persistent antiphospholipid antibodies (aPLs), thrombosis, recurrent abortion, complications during pregnancy, and occasionally thrombocytopenia. The objective of the present study was to review the pathophysiology of APS and its association with female infertility. A bibliographic review of articles of the past 20 years was performed at the PubMed, Scielo, and Bireme databases. Antiphospholipid antibody syndrome may be associated with primary infertility, interfering with endometrial decidualization and with decreased ovarian reserve. Antiphospholipid antibodies also have direct negative effects on placentation, when they bind to the trophoblast, reducing their capacity for invasion, and proinflammatory effects, such as complement activation and neutrophil recruitment, contributing to placental insufficiency, restricted intrauterine growth, and fetal loss. In relation to thrombosis, APS results in a diffuse thrombotic diathesis, with global and diffuse dysregulation of the homeostatic balance. Knowing the pathophysiology of APS, which is closely linked to female infertility, is essential for new therapeutic approaches, specialized in immunomodulation and inflammatory signaling pathways, to provide important advances in its treatment.


Assuntos
Síndrome Antifosfolipídica , Infertilidade Feminina , Aborto Habitual , Adulto , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/fisiopatologia , Feminino , Humanos , Infertilidade Feminina/complicações , Infertilidade Feminina/fisiopatologia , Pessoa de Meia-Idade , Gravidez
9.
JNMA J Nepal Med Assoc ; 57(216): 133-145, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31477950

RESUMO

Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific antibodies against phospholipid binding plasma proteins in the serum of the patient, with or without underlying autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for diagnosis of Anti-phospholipid Antibody Syndrome. Stroke or myocardial infarction in young, unprovoked recurrent deep vein thrombosis and recurrent pregnancy loss are typical scenarios where Anti-Phospholipid Antibody Syndrome should be suspected. Presence of non-criteria manifestations like livedo reticularis, skin ulcers, nephropathy, valvular heart disease and thrombocytopenia adds to diagnostic clue for presence of Anti-Phospholipid Antibody Syndrome. Treatment of Anti-Phospholipid Antibody Syndrome has preventive and therapeutic aspects that usually focus on thrombotic and obstetric manifestations of the disease. Therapeutic anti-coagulation with heparin followed by warfarin is required for patients presenting with acute thrombosis. Those with venous thrombosis are given moderate intensity warfarin International Normalized Ratio, 2-3), whereas those with arterial thrombosis or recurrent venous thrombosis even on warfarin are treated with high intensity warfarin (International Normalized Ratio, 3-4). Similarly, anticoagulation with heparin is advised in patients with obstetric Anti-Phospholipid Antibody Syndrome throughout pregnancy and up to six weeks postpartum. Treatment recommendations are still not clear for asymptomatic Anti-Phospholipid Antibody Syndrome positive patients and in those with non-criteria manifestations of the disease. Steroids, intravenous immunoglobulin and immunosuppressant are reported to be effective in severe cases of catastrophic antiphospholid syndrome characterized by rapid small vessel thrombotic involvement of multiple organ systems. Studies are evaluating the efficacy of direct thrombin inhibitors in the management of refractory cases. Keywords: anticoagulants; anti-phospholipid syndrome; obstetric APS; thrombotic APS.


Assuntos
Síndrome Antifosfolipídica/terapia , Trombose/etiologia , Anticorpos Antifosfolipídeos/imunologia , Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/fisiopatologia , Feminino , Humanos , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/fisiopatologia , Complicações na Gravidez/terapia , Trombose/tratamento farmacológico
11.
Brain Dev ; 41(6): 555-558, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30857880

RESUMO

Infarct locations in children with arterial ischemic stroke have primarily been reported to be lobar or in the basal ganglia, and those in patients with Down syndrome (DS) and antiphospholipid syndrome (APS) are typically wide and multiple. No solitary brain stem infarctions have ever been reported in children with DS until now. Here, we report a case of brain stem infarction in a 6-year-old boy with DS who had no cardiac, renal, or intestinal complications. He exhibited ataxic gait and medial longitudinal fasciculus (MLF) symptoms at first presentation. Neuroimaging revealed a localized and isolated lesion in the midbrain. Although he did not satisfy the diagnostic criteria of APS, he showed persistently elevated levels of anticardiolipin antibody (21 U/mL; normal value <10 U/mL). Although he had the risks of a multiple vascular systems disorder, DS, and persistently elevated levels of antiphospholipid antibodies, his lesion was not similar to any of the previously reported cerebral infarctions in DS or in APS. To our knowledge, this is the first report of limited solitary brain stem infarction in a child with DS.


Assuntos
Infartos do Tronco Encefálico/fisiopatologia , Síndrome de Down/complicações , Anticorpos Anticardiolipina/análise , Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/fisiopatologia , Tronco Encefálico/fisiopatologia , Infartos do Tronco Encefálico/metabolismo , Infarto Cerebral/fisiopatologia , Criança , Síndrome de Down/fisiopatologia , Humanos , Infarto/fisiopatologia , Japão , Masculino
12.
Lupus ; 28(4): 483-491, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30755144

RESUMO

INTRODUCTION: Transcranial Doppler is a method that enables the assessment of different cerebral hemodynamic parameters. It also allows for the evaluation of the presence of right-to-left circulation shunts (RLS) and for the detection of microembolic signals (MESs), which might be associated with an increased risk of cerebrovascular events. For instance, the presence of MESs on transcranial Doppler in patients with systemic lupus erythematous (SLE) and antiphospholipid syndrome (APS) is associated with an increased risk of stroke. Therefore, transcranial Doppler could be a useful tool for stroke risk stratification in these patients. OBJECTIVE: Our objective was to evaluate transcranial Doppler cerebral mean blood flow velocities as well as the presence of MESs and RLS in patients with antiphospholipid syndrome and SLE. PATIENTS AND METHODS: Twenty-two patients with primary APS (PAPS), 24 patients with secondary APS (SAPS), 27 patients with SLE without APS and 21 healthy controls were evaluated. Clinical and epidemiological data were compiled from medical charts, and all subjects underwent transcranial Doppler examination with breath-holding index calculation. Both middle cerebral arteries were monitored for 60 min for the detection of MESs. RLS was investigated with agitated saline injected as a bolus. RESULTS: There were no significant differences in mean blood flow velocities among the groups. MESs were more frequently found in patients with SLE when compared with controls and patients with APS (SLE: 17.4%, SAPS: 4.3%, PAPS: 0%, controls: 0%, p = 0.03). Anticoagulant therapy was more frequently used in the APS group (PAPS: 81.8%, SAPS: 75.2%, SLE: 1.7%, p < 0.001). Patients with APS had a higher frequency of RLS when compared with volunteers (63.6% versus 38.1%, p = 0.05). Breath-holding index values tended to be lower in patients with SAPS than in control subjects and patients with PAPS and SLE ( p = 0.06). CONCLUSIONS: Patients with APS had a higher frequency of RLS than healthy controls. This finding alerts to the importance of cardiac investigation in patients with stroke and APS, because further therapies such as RLS occlusion might eventually add protection. The higher frequency of MES in patients with SLE could suggest an effect of anticoagulant therapy on MES prevention, more frequently used in patients with APS.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico por imagem , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico por imagem , Lúpus Eritematoso Sistêmico/fisiopatologia , Ultrassonografia Doppler Transcraniana , Adulto , Síndrome Antifosfolipídica/fisiopatologia , Artérias/diagnóstico por imagem , Autoanticorpos/análise , Contagem de Células Sanguíneas , Testes de Coagulação Sanguínea , Velocidade do Fluxo Sanguíneo , Encéfalo/irrigação sanguínea , Infarto Encefálico/etiologia , Suspensão da Respiração , Circulação Cerebrovascular , Feminino , Humanos , Hipertensão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Risco , Fatores de Risco , Estatísticas não Paramétricas , Trombose/fisiopatologia
13.
Intern Emerg Med ; 14(4): 521-527, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30603858

RESUMO

How thrombophilia may contribute to the development of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. We searched on PubMed and EMBASE (until 15 April 2018), studies on CTEPH reporting data on inherited or acquired thrombophilia. Starting from 367 articles mentioning the search terms, 347 were excluded mainly as duplicate articles or articles not in English. After reading the full text of remaining articles, ten were excluded for being reviews, editorials, letters or case reports, and two were further removed from the analysis because of the potential selection bias. All the eight considered studies provided the proportion of patients positive for antiphospholipid (aPL) antibodies. The crude rate of aPL in CTPEH patients is 11.8% (95% CI 10.09-13.8%). The meta-analysis considering the weighted mean proportion and 95% confidence intervals (CIs) yields a rate of aPL antibody-positive profile of 12.06% (95% CI 8.12-16.65%) among the patients with CTEPH in the random effects model (I2 76.33%; 95% CI 52.75-88.14%, p = 0.0001). The sensibility analysis confirms the result. No predictors of heterogeneity are found in a meta-regression analysis. Our results suggest that aPL antibodies are frequently associated with CTEPH underlining the need to test for aPL antibodies in young patients with "idiopathic" and "provoked" PE caused by mild provoking risk factors.


Assuntos
Anticorpos Antifosfolipídeos/análise , Hipertensão Pulmonar/complicações , Prevalência , Trombose/etiologia , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/fisiopatologia , Humanos , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/fisiopatologia , Fatores de Risco , Trombose/sangue , Trombose/fisiopatologia
14.
J Clin Lab Anal ; 33(1): e22617, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29992646

RESUMO

INTRODUCTION: The patients with antiphospholipid syndrome (APS) associate an increased risk of atherosclerosis. OBJECTIVE: To determine the predictors of an abnormal ankle-brachial index (ABI), surrogate measure of atherosclerosis, in patients with APS. METHODS: The ABI was measured according to standard recommendations in 106 patients. Traditional cardiovascular risk factors were assessed in all cases. A large spectrum of APS antibodies was determined in 73 patients. RESULTS: A total of 106 patients diagnosed with APS were included. 28.3% patients included were found to have low ABI. Anti-beta 2-glycoprotein I (aß2GPI) IgG antibodies [4.00 (1.00-79.00) vs 3.00 (0.00-29.00) U/mL, P = 0.02] and antiprothrombin (aPT) IgM antibodies [4.50 (0.00-82.00) vs 3.00 (0.00-14.00) U/mL, P = 0.05] titers were found to be higher in patients with abnormal ABI. However, after multivariate regression analysis, only the aß2GPI IgG titer remained predictor of low ABI (P = 0.04). CONCLUSIONS: aß2GPI IgG associated with impaired ABI in patients with APS. This relation might reflect their involvement in the atherosclerosis occurrence.


Assuntos
Índice Tornozelo-Braço/estatística & dados numéricos , Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/fisiopatologia , Adulto , Aterosclerose , Autoanticorpos/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Risco , beta 2-Glicoproteína I/imunologia
15.
G Ital Dermatol Venereol ; 154(3): 277-285, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30375212

RESUMO

Antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition characterized by the onset of venous and/or arterial thrombosis, often multiple, and pregnancy morbidity in a background of antiphospholipid antibodies (aPL) positivity. Some patients can be carrier of aPL with no clinical symptoms, in other cases clinical manifestation can range from the classical presentation to an acute life-threatening condition named catastrophic APS. APS can be considered as primary or associated to other disease, however pregnancy acts as a triggering factor on a susceptible background that lead to the clinical manifestations through immunological and non-immunological mechanism. APS is characterized by thrombotic manifestation involving both venous and arterial vessels of different size, in any part of the body the clinical presentation can be extremely polymorphic. Livedo reticularis is the most common cutaneous finding, however it is not specific for APS and other cutaneous manifestation such as ulcers or purpura can be the only presentation of APS. Diagnosis is established by clinical and laboratory criteria. Counselling, pregnancy plan and monitoring during pregnancy is mandatory in APS patients. Treatment can be challenging, and it is mainly based on the use of anticoagulant and antiaggregant medications. A literature review on PubMed about APS and pregnancy was performed, and the significant information in conjunction with our clinical experience lead to the drafting of this paper. We present a review of APS and its management in pregnancy, with a special focus on the dermatological presentation and the role of the dermatologists in diagnosing and managing this potential life-threatening condition.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/terapia , Complicações na Gravidez/terapia , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/fisiopatologia , Feminino , Humanos , Livedo Reticular/etiologia , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/fisiopatologia , Trombose/etiologia
16.
Biosystems ; 177: 44-47, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30389556

RESUMO

The paper shows that osteoporosis (OA) changes the SF content and the lipid profile substantially. To estimate the implication of the lipid environment in case the articular cartilage (AC) changes, we measured friction coefficient normal samples, with early and late stages of (OA). During joint inflammation and osteoarthritis, enzymatically activated ß2-Glycoprotein I is transformed into antibody conformation. Our hypothesis about cartilage degradation of PL bilayers by antibodies (ß2-Glycoprotein I) is considering antiphospholipid syndrome (APS), which was not discussed in the literature before. Deactivated PL molecule has no ability to form bilayers, lamellar phases, and liposomes. The phospholipid content in synovial fluid (SF) during joint inflammation, osteoarthritis, and rheumatoid arthritis is significantly higher (2-3 times) above the normal concentration of PL, and has a poor boundary-lubricating ability is deactivated.


Assuntos
Síndrome Antifosfolipídica/fisiopatologia , Artrite/fisiopatologia , Cartilagem Articular/fisiopatologia , Articulação do Joelho/fisiopatologia , Lipídeos/análise , Líquido Sinovial/metabolismo , Animais , Estudos de Casos e Controles , Bovinos , Fricção , Humanos , Lubrificação , Modelos Biológicos
17.
Rheumatol Int ; 39(3): 533-539, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30415452

RESUMO

Systemic lupus erythematosus (SLE), pathology with net feminine predominance, is one of the most complex autoimmune diseases and has major impact on patients' life. The aim is to identify patient and disease-related factors associated with self-perceived disease severity in female SLE patients. This cross-sectional study enrolled 73 women fulfilling the 2012 Systemic Lupus International Collaborating Clinic (SLICC) criteria. SLE disease activity was assessed by the Systemic Lupus Activity Measure (SLAM) score and overall damage by the SLICC/American College of Rheumatology (ACR) index. Patients' general characteristics, associated conditions as well as SLE specific clinical involvements and therapeutic principles were also noted. Fatigue was assessed by FACIT-fatigue scale. Self-perceived disease severity was assessed using numerical rating scales (1-10 NRSs), to evaluate the disease severity at inclusion (1-10 NRS now) and worst severity anytime during disease history (1-10 NRS worst ever). In regard to worst ever lupus severity, 54.8% of patients responded with 9 or 10, while none with 1 or 2 even if only 22.9% of the patients responded with 7 or more for disease severity at inclusion (1-10 NRS now). Women with higher 1-10 NRS now answers had also higher 1-10 NRS worst ever, SLAM, SLICC, and FACIT-fatigue scores. They associated more frequently anxiety/depression diagnosis, antiphospholipid syndrome, joint involvement as well as treatments with corticosteroids. Self-reported disease severity worst ever, anxiety/depression diagnosis, fatigue, and the daily dose of corticosteroids were independently associated with patients' perception on lupus severity at inclusion: OR (95% CI), 2.13 (1.15-3.94) p = 0.017, 6.67 (1.11-39.97) p = 0.038, 1.10 (1.02-1.19) p = 0.018, and 1.11 (1.02-1.21) p = 0.020, respectively. The vast majority of patients identified severe and very severe events during their disease history, results that raise awareness of burden concerning lupus occurrence in women's life. Self-perceived lupus severity is multifactorial, influenced also by factors less considered in the SLE management like fatigue and the depression/anxiety disorders, but also by the previous patient's experience.


Assuntos
Corticosteroides/uso terapêutico , Síndrome Antifosfolipídica/fisiopatologia , Fadiga/fisiopatologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Adulto , Síndrome Antifosfolipídica/complicações , Transtornos de Ansiedade/complicações , Transtornos de Ansiedade/psicologia , Estudos Transversais , Transtorno Depressivo/complicações , Transtorno Depressivo/psicologia , Fadiga/etiologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/psicologia , Pessoa de Meia-Idade , Autorrelato , Índice de Gravidade de Doença
18.
Lupus ; 27(1_suppl): 28-31, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30452326

RESUMO

Antiphospholipid syndrome (APS) covers a spectrum of clinical manifestations ranging from recurrent pregnancy loss and obstetric complications from placental dysfunction through to thrombotic disease. This article will focus on the common manifestations of the pregnancy-related complications of APS. This includes clinical manifestations, diagnosis and management, as general practitioners will need to be able to recognize the disorder and will also have patients under their care receiving treatment for APS.


Assuntos
Aborto Habitual/etiologia , Síndrome Antifosfolipídica/diagnóstico , Complicações na Gravidez/diagnóstico , Resultado da Gravidez , Aborto Habitual/diagnóstico por imagem , Adulto , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/fisiopatologia , Aspirina/uso terapêutico , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Gravidez , Complicações na Gravidez/tratamento farmacológico , Complicações na Gravidez/fisiopatologia
20.
Int J Cardiol ; 271: 366-370, 2018 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-30223375

RESUMO

BACKGROUND: Antiphospholipid syndrome (APS) can be primary or secondary to other autoimmune disorders. Besides valvular heart disease (VHD) and coronary artery disease (CAD), little is known about the impact of APS on left ventricular (LV) function. METHODS: After excluding CAD, relevant VHD and heart failure, 69 patients (mean age = 43.9 years, 40 with primary and 29 with secondary APS) were assessed by echo-Doppler. Sixty-nine heathy controls, matched for age and sex, formed the control group. APS was diagnosed in presence of at least one clinical criteria and one confirmed laboratory criteria, including lupus anticoagulant (LA) titre. The adjusted global APS score (aGAPSS), derived from the combination of risk factors for thrombosis and autoimmune-antibody profile was calculated. RESULTS: Patients had similar blood pressure and heart rate, but higher body mass index (BMI) than controls. LV mass index (p = 0.007) and left atrial volume index (p < 0.01) were greater, while early diastolic velocity (e') was lower (p = 0.003) and E/e' higher (p = 0.007) in APS. Primary APS patients had lower E/A and e' compared to both controls and secondary APS, while E/e' was higher in secondary APS than in controls. APS patients with diastolic dysfunction were older but did not differ for risk factors prevalence from those with normal/indeterminate diastolic function. In the pooled APS, LA positivity was independently associated with e' and E/e' after adjusting for age, BMI and aGAPSS in separate multivariate models. CONCLUSION: In APS, LV diastolic abnormalities are detectable. They are more pronounced in primary APS and independently associated with LA positivity.


Assuntos
Síndrome Antifosfolipídica/diagnóstico por imagem , Ecocardiografia Doppler/métodos , Ecocardiografia/métodos , Doenças das Valvas Cardíacas , Disfunção Ventricular Esquerda/diagnóstico por imagem , Adulto , Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/fisiopatologia
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