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1.
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
3.
Beijing Da Xue Xue Bao Yi Xue Ban ; 51(6): 1178-1181, 2019 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-31848526

RESUMO

A 58-year-old female was referred to our department with intermittent suffocation for 1.5 years, aggravated for a month. 1.5 years before she developed oral ulcer, raynaud phenomenon, proteinuria, bilateral pleural effusion, ANA and anti-dsDNA positive. This patient was diagnosed with systemic lupus erythematosus (SLE). After given hormones, hydroxychloroquine sulfate (HCQ), her symptom relieved soon. The patient stopped her pills 1 year ago. One month ago, she had chest tightness, increased urine foam, and suffered from oliguria. Her admission medical examination: blood pressure (BP) 130/80 mmHg, conjunctiva pale, and lower lung breath sounds reduced. There was no tenderness, rebound pain and abdominal muscle tension in the abdomen. Liver and spleen rib inferior, mobile dullness negative, and lower extremity edema. Blood routine tests were performed with hemoglobin (HGB) 57 g/L. Urine routine: BLD (3+). 24-hour urinary protein 3.2 g. serum albumin 20.5 g/L, C-reactive protein (CRP) 12.85 mg/L, erythrocyte sedimentation rate (ESR) 140 mm/h. Antinuclear antibody (ANA) (H)1:10 000, anti-dsDNA antibody 1:3 200; anti-Smith antibody, anti-U1-snRNP/Sm antibody were positive, blood complement 3(C3) 0.43 g/L, complement 4(C4) 0.07 g /L. Anticardiolipin antibody (ACL), anti-ß2-GP1, lupus anticoagulant (LA) were negative; HRCT suggested bilateral medial pleural cavity product liquid. Admission diagnosis: SLE lupus nephritis, anemia, pleural effusion, and hypoproteinemia. We treated her with methylprednisolone 1 000 mg×3 d, late to 48 mg/d and cyclophosphamide 1.0 g, HCQ 0.2 g bid, gamma globulin 10 g×5 d. Day 2 of treatment, this patient developed acute right upper quadrant pain, not accompanied by nausea, vomiting, blood stool and diarrhea. Antipyretic antispasmodic treatment was invalid, after the morning to ease their own abdominal pain. Day 4 of treatment, daytime blood HGB 77 g/L. Bilateral renal vascular ultrasound: bilateral renal artery blood flow velocity was reduced. The abdominal pain of the above symptoms recurred at night, BP was 120/80 mmHg, and no positive signs were found on abdominal examination. No abnormality was found in the vertical abdominal plain film. Blood routine examination: HGB 53 g/L, Plasma D dimer 2 515 µg/L, amylase in hematuria was normal, the stool occult blood was negative. Abdominal computed tomography (CT): normal structure of right adrenal gland disappeared, irregular mass shadow could be seen in adrenal region, CT value was about 50 HU. Morphological density of left adrenal gland was not abnormal. The retroperitoneum descended along the inferior vena cava to the right iliac blood vessel and showed a bolus shadow. The density of some segments increased. The lesion involved the right renal periphery and reached the left side of abdominal aorta. Most lesions surrounded the inferior vena cava, the right renal vein and part of the small intestine. The boundary between the upper lesion and the vena cava was unclear. Iodinecontaining contrast agent was taken orally. No sign of contrast agent overflowing was found in the abdominal cavity. Hematoma and exudative changes were considered in retroperitoneum. CONCLUSION of contrast-enhanced ultrasound of blood vessels: The retroperitoneal inferior vena cava (volume 3.5 cm×3.5 cm×1.5 cm) was hypoechoic and had no blood flow lesion. The adrenal gland had a high possibility of origin. Left renal vein thrombosis extended to inferior vena cava. According to the above data, it was analyzed that the cause of retroperitoneal hematoma of the patient was left adrenal vein thrombosis caused by hypercoagulable state, which led to vascular rupture and hemorrhage caused by increased vascular pressure in adrenal gland. Therefore, on the basis of continuing to actively treat the primary disease, and on the basis of dynamic observation of no active hemorrhage for 3 days, the anticoagulant therapy was continued with 10 mg/d of apixaban. Clinical symptoms were gradually eased, HGB did not decrease. Two weeks later, the ultrasonic examination showed that the irregular cluster hypoechoic range behind the inferior vena cava was significantly smaller than that before (1.8 cm×1.2 cm×0.7 cm). Abdominal CT examination after 1 month showed that there was no abnormal morphological density of bilateral adrenal glands and basic absorption of retroperitoneal exudation. Adrenal hemorrhage is uncommon. SLE with adrenal hemorrhage is rarer. In SLE patients, especially those complicated with APS, if abdominal pain accompanied by HGB decrease occurs, except after gastrointestinal hemorrhage, the possibility of adrenal hemorrhage should be warned.


Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Anticorpos Anticardiolipina , Aorta Abdominal , Feminino , Hemorragia , Humanos , Pessoa de Meia-Idade
4.
Kardiologiia ; 59(12): 92-96, 2019 Dec 11.
Artigo em Russo | MEDLINE | ID: mdl-31849316

RESUMO

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology characterized by a wide range of clinical manifestations with damage to various organs and systems of the body. There are bad prognostic factors for SLE: damage to the heart, kidney, central nervous system, the development of hematological crises and secondary antiphospholipid syndrome. A number of authors consider systemic lupus erythematosus a "new" risk factor for atherosclerosis. The overall risk of myocardial infarction (MI) in patients with SLE is 10 times higher than in the general population. The article presents clinical case report of the development of myocardial infarction in a woman with SLE, receiving therapy for secondary antiphospholipid syndrome.


Assuntos
Síndrome Antifosfolipídica , Aterosclerose , Lúpus Eritematoso Sistêmico , Infarto do Miocárdio , Feminino , Humanos , Fatores de Risco
5.
Zhonghua Nei Ke Za Zhi ; 58(12): 894-898, 2019 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-31775452

RESUMO

Objective: Portal vein thrombosis (PVT) is a rare and severe clinical manifestation of antiphospholipid syndrome (APS), as well as a predictor of poor prognosis. This study was conducted to explore the clinical features and risk factors of PVT in APS patients. Methods: A total of 123 APS patients diagnosed from 2012 to 2019 were retrospectively enrolled. The diagnosis of PVT was made according to the 2009 American College of Liver Diseases (AASLD) criteria. Clinical and laboratory data were collected. A multivariate (MV) logistic regression model was constructed using a stepwise forward selection procedure among those candidate univariables with P values<0.10. Results: A total of 28 cases with PVT, and 95 control cases without PVT were finally enrolled.The 28 APS-PVT patients included 5 males and 23 females with age range from 17 to 63 years. Clinical manifestations included acute thrombosis in 8 patients, chronic thrombosis in 16, and 4 with portal vein spongiform. As to the involved vessels, single portal vein thrombosis was seen in 20 patients, portal combined with superior mesenteric vein (SMV) and splenic vein in one patient, portal plus SMV in 4 and only SMV in 3 patients. Other manifestations were portal hypertension (16/28), esophageal varices (13/28), spleen infarction (7/28) and gastrointestinal bleeding (4/28). Two antiphospholipid antibodies were positive in 13 cases. Triple positive antibodies were seen in 7 cases. Multivariate logistic regression analysis showed that disease duration less than 0.5 years (OR=72.74, 95%CI 7.50-705.45, P<0.001), hypoalbuminemia (OR=356.45, 95%CI 19.19-6 620.14, P<0.001), and elevated erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) (OR=14.41, 95%CI 1.49-139.20, P<0.001) were independent risk factors for PVT in APS. Conclusion: PVT is usually misdiagnosed due to insidious onset. Short disease duration, hypoalbuminemia and elevated ESR/CRP are risk factors for PVT in APS. Better understanding, early diagnosis and treatment will improve the clinical outcome.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/imunologia , Cirrose Hepática/imunologia , Veia Porta/patologia , Trombose/imunologia , Trombose Venosa/fisiopatologia , Adolescente , Adulto , Síndrome Antifosfolipídica/complicações , Feminino , Humanos , Cirrose Hepática/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Trombose/complicações , Trombose Venosa/complicações , Adulto Jovem
6.
Biochemistry (Mosc) ; 84(9): 992-1007, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31693459

RESUMO

The review discusses the mechanisms of participation of natural killer T cells (NKT cells) in the induction of antiphospholipid antibodies (APA) that play a major pathogenetic role in the formation of antiphospholipid syndrome (APS), summarizes the data on APS pathogenesis, and presents modern concepts on the antibody formation involving follicular helper type II NK cells.


Assuntos
Síndrome Antifosfolipídica/imunologia , Células T Matadoras Naturais/imunologia , Animais , Anticorpos Antifosfolipídeos/imunologia , Humanos
7.
Klin Lab Diagn ; 64(10): 603-606, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31742953

RESUMO

The objective of the study is to enhance sorption capacity of diagnostic agents by using cardiolipin antigens for antiphospholipid syndrome in patients with systemic lupus erythematosus (SLE). A technique of emulsion polimerization was used. Having integrated antigen nanoobjects we developed immobilized magnetocontrollable antigen nanosystems and put them to an evaluation test. The nanosystems are polyacrylamide granules with a built in antigen. To obtain stable immobilized multi-use biopharmaceuticals with targeted properties (shape, particle diameter, pore size, density) we used a modified version of emulsion polymerization method using polyacrylamide carrier gel. This method permitted a greater sorptive capacity, preserving the antigen in maximum native state, and opened up the possibility of controllable modification of nanoobjects. Cardiolipin was used as the antigen in question. Following the method described above we performed sorption of anticardiolipin antibodies from blood plasma of SLE patients who showed clinical presentations of antiphospholipid syndrome. All SLE patoents with signs of antiphospholipid syndrome showed reliably higher levels of cardiolipin antibodies compared with SLE patients without antiphospholipid syndrome signs; the antibody level was 0.365 ± 0.026 and 0.075 ± 0.003 on average, correspondingly (p < 0.001). Blood serum from 10 apparently healthy individuals served as control. The level of cardiolipin antibodies was determined before and after sorption by indirect solid phase immunoenzyme method. In the eluate we estimated total protein by Lowry method. In vitro testing showed that the obtained antigen nanosystems based on immobilized cardiolipin could effectively remove cardiolipin antibodies from whole blood of SLE patients with clinical presentations of APS to achieve the values of healthy individuals (before sorption cardiolipin antibodies 0.328 ± 0.0289; after sorption 0.059 ± 0.0170; p<0,001; sorption capacity 8.00 ± 0.390 mg/ml). The method of emulsion polymerization with consideration to hydrophobic and hydrophilic properties of lipid molecules permits obtaining and modifying biomolecules with certain properties, in a controlled fashion.


Assuntos
Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Síndrome Antifosfolipídica/complicações , Humanos , Lipídeos , Nanotecnologia
8.
Presse Med ; 48(11 Pt 2): 347-353, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31694791

RESUMO

Plasma exchange is a well-established therapeutic procedure commonly used in many autoimmune disorders. The beneficial effects of plasma exchange are thought to occur through the elimination of pathogenic mediators found in plasma, including autoantibodies, complement components, and cytokines. The catastrophic antiphsopholipid syndrome (CAPS) is a life-threatening variant of the antiphospholipid syndrome (APS) where several thrombosis take place in a short period of time in patients with circulating antiphospholipid antibodies. The triple therapy with anticoagulation, corticosteroids and plasma exchange or intravenous immunoglobulins has been proposed in CAPS. CAPS is a rare disease precluding the conduction of formal clinical trials. However, the observation of a better clinical course of patients who received this treatment supports their use. Plasma exchange has become an established therapeutic procedure in CAPS but there are no studies regarding the better approach and thus its use relies on the experience of the physicians in charge. The current article aims to review potential mechanisms of action of plasma exchange and the technical aspects of this procedure and will focus on its current role in CAPS, the experience published in treating this condition and the treatment protocol that we use in our institution.


Assuntos
Síndrome Antifosfolipídica/terapia , Troca Plasmática/métodos , Corticosteroides/uso terapêutico , Anticorpos Antifosfolipídeos/sangue , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Terapia Combinada/métodos , Hidratação/métodos , Humanos
10.
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
11.
J Clin Neurosci ; 70: 247-249, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31540859

RESUMO

We present a case demonstrating histopathological clot findings after endovascular thrombectomy for acute ischaemic stroke in a 38 year-old male with systemic lupus erythematosus and antiphospholipid syndrome (APS). The differential diagnosis was embolism of a suspected Libman-Sacks vegetation or less likely an in-situ thrombosis. Clot analysis provided guidance with patient management and anticoagulation was commenced. The utility of clot analysis in this case provides support for routine clot analysis, which has been standard practice at our institution, and is likely to evolve as endovascular thrombectomy becomes more widely accessible.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Acidente Vascular Cerebral/etiologia , Trombose/patologia , Adulto , Procedimentos Endovasculares , Humanos , Masculino , Acidente Vascular Cerebral/cirurgia , Trombectomia
13.
Vasc Health Risk Manag ; 15: 253-258, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31496715

RESUMO

Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia characterized by venous and/or arterial thromboses, pregnancy morbidity (predominantly repeated fetal losses), and the presence of phospholipid antibodies. The estimated annual incidence of APS is 5 new cases per 100,000 people. The most common thrombotic events in patients with APS in order of frequency are stroke, transient ischemic attack, deep vein thrombosis, and pulmonary embolism. Patients with APS may develop an intracardiac thrombus, which is a life-threatening complication with a high risk of increased morbidity and mortality; however, it is treatable by surgical removal, extensive anticoagulant administration, and prevention of other complications. Catastrophic APS, which is a rare and severe condition diagnosed based on rapidly progressive thromboembolic events involving three or more organs, systems, or tissues, occurs in less than 1% of all patients with APS. We herein report an autopsy case of catastrophic APS in a 12-year-old Thai boy with multiple thromboembolic events including intracardiac thrombus formation with a positive lupus anticoagulant test result. To the best of our knowledge, this is the youngest reported patient with APS to date.


Assuntos
Síndrome Antifosfolipídica/complicações , Cardiopatias/etiologia , Tromboembolia/etiologia , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Autopsia , Biomarcadores/sangue , Doença Catastrófica , Criança , Evolução Fatal , Cardiopatias/sangue , Cardiopatias/diagnóstico , Cardiopatias/terapia , Humanos , Inibidor de Coagulação do Lúpus/sangue , Masculino , Tromboembolia/sangue , Tromboembolia/diagnóstico , Tromboembolia/terapia
14.
Artigo em Russo | MEDLINE | ID: mdl-31464289

RESUMO

AIM: To study clinical characteristics and Doppler and laboratory parameters in women with headache and antiphospholipid syndrome. MATERIAL AND METHODS: One hundred and forty-three women with headache (34 with migraine, 53 with tension type headache) and antiphospholipid syndrome and 56 healthy controls were examined. RESULTS: Coagulogram and cerebral blood flow parameters were significantly different between controls and patients with headache and antiphoshpolipid syndrome. All women with antiphoshpolipid syndrome had changes in the thrombotic system and plasma. Blood flow parameters were higher in all patients with headache and antiphoshpolipid syndrome. CONCLUSION: Changes in laboratory and Doppler parameters depend on the clinical manifestations of cephalalgia, as well as the age of patients.


Assuntos
Síndrome Antifosfolipídica , Transtornos de Enxaqueca , Cefaleia do Tipo Tensional , Síndrome Antifosfolipídica/diagnóstico por imagem , Feminino , Cefaleia/diagnóstico por imagem , Humanos , Transtornos de Enxaqueca/diagnóstico por imagem , Cefaleia do Tipo Tensional/diagnóstico por imagem , Ultrassonografia Doppler
15.
Neurosciences (Riyadh) ; 24(3): 240-244, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31380826

RESUMO

New-onset refractory status epilepticus (NORSE) is a drug-resistant status epilepticus that often has a catastrophic outcome. Our patient was diagnosed with NORSE and had an EEG reading that showed status epilepticus persisting for 8 months in general anesthesia. After autoimmune workup showed positive antiphospholipid antibodies, his seizure was controlled, and he was discharged with good condition apart from moderate cognitive impairment. However, he later developed schizophrenia. Although psychiatric disorders have been associated with antiphospholipid syndrome, to the best of our knowledge, it has not been reported to be associated with status epilepticus. We recommend vigilance of psychological complications of refractory status epilepticus patients for early psychiatric referral, diagnosis, and treatment.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Esquizofrenia/diagnóstico , Estado Epiléptico/diagnóstico , Adulto , Síndrome Antifosfolipídica/complicações , Humanos , Masculino , Esquizofrenia/etiologia , Estado Epiléptico/etiologia
16.
Autoimmun Rev ; 18(9): 102352, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31323355

RESUMO

Studies on last genetic and epigenetic predisposition to APS are summarized. It is well known that genetic predisposition is in HLA system (DR4 and DRw53) and that lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) are both associated with the same HLA antigens. Other genes, outside the MHC, give their contribution to the development of this autoimmune syndrome, such as IRF5, STAT4 and those related to inherited thrombophilia - factor V Leiden and G20210A prothrombin polymorphisms. Finally, post-transcriptional modifications of anti-beta2GPI antibodies could be implicated too. The most important discovery of last years is that altered microRNAs' expression is linked to autoimmunity, thrombosis, early atherosclerosis, and oxidative stress in APS.


Assuntos
Síndrome Antifosfolipídica/genética , Epigênese Genética/fisiologia , Antígenos HLA/genética , Resistência à Proteína C Ativada/sangue , Resistência à Proteína C Ativada/genética , Resistência à Proteína C Ativada/imunologia , Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Predisposição Genética para Doença , Genótipo , Humanos , Inibidor de Coagulação do Lúpus/sangue , Polimorfismo Genético , Trombose/complicações , Trombose/genética , Trombose/imunologia , beta 2-Glicoproteína I/imunologia
17.
Lupus ; 28(10): 1181-1188, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31345117

RESUMO

Antiphospholipid syndrome an autoimmune disease characterized by thrombosis and/or pregnancy morbidity alongside the presence of antiphospholipid antibodies (aPL). This review evaluates primary and secondary thromboprophylaxis in patients with aPL and thrombotic events. In primary thromboprophylaxis a risk-stratified approach is needed based on aPL, comorbidity with other autoimmune conditions and cardiovascular vascular risk factors. In primary thromboprophylaxis, the efficacy of low-dose aspirin is debatable and requires better-designed controlled studies. So far warfarin has not been shown to improve venous and/or arterial thrombosis incidence in aPL carriers and instead increased safety concerns. The benefit of hydroxychloroquine is inconclusive despite promising data, requiring large, controlled trials. For secondary thromboprophylaxis warfarin seems to be the best option with potential in renal transplant recipients and better efficacy at high intensity, although maintenance of target international normalized ratio needs careful monitoring. Aspirin has not shown to be beneficial, and data on rivaroxaban are limited and contradictory. Despite all data being informative, there are limitations that need to be addressed with robust clinical trials.


Assuntos
Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/complicações , Trombose/prevenção & controle , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/imunologia , Doenças Autoimunes/complicações , Humanos , Coeficiente Internacional Normatizado , Fatores de Risco , Trombose/etiologia
18.
Lupus ; 28(9): 1158-1166, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31299879

RESUMO

INTRODUCTION: Although extensively characterized in the outpatient setting, systemic lupus erythematosus (SLE) in the hospitalization wards is still scarcely portrayed, particularly in the perspective of its evolution over the years. METHODS: Retrospective analysis of SLE patients hospitalized in the Department of Autoimmune Diseases of a university hospital during a 20-year period (1995-2015), describing hospitalization characteristics, causes and predictors of outcome. RESULTS: A total of 814 hospitalizations concerning 339 patients were analysed. The main causes of admission were flare (40.2%), infection (19.2%), diagnostic procedures (18.8%) and thrombotic events (5.4%). Therapy with cyclophosphamide (odds ratio (OR) 1.908, p = 0.047) was associated with admission due to infection, while antimalarials displayed a protective effect (OR 0.649, p = 0.024). Nearly 3.9% of patients required admission to an intensive care unit, with associated antiphospholipid syndrome (OR 7.385, p = 0.04) standing as a predicting factor for this outcome. Readmission at 30 days occurred in 5.8% of patients, with thrombocytopenia (OR 6.007, p = 0.002) and renal involvement (OR 3.362, p = 0.032) featuring as predicting factors. Eight patients died, with antiphospholipid syndrome (OR 26.814, p = 0.02) and thrombocytopenia (OR 31.523, p = 0.01) being associated with mortality. There was no significant variation in patients' demographics or admission causes across the 20-year period, except for a decrease in admissions due to thrombotic and musculoskeletal causes. Recently, an increase in the use of mycophenolate mofetil and lower doses of glucocorticoids were noted. CONCLUSION: While demographics of SLE hospitalizations have not markedly changed over the past 20 years, changes in therapy patterns were observed. Thrombocytopenia, antiphospholipid syndrome and renal involvement featured as predictors of poor outcome.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/terapia , Trombocitopenia/epidemiologia , Adulto , Síndrome Antifosfolipídica/etiologia , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/mortalidade , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Readmissão do Paciente/estatística & dados numéricos , Estudos Retrospectivos , Espanha , Trombocitopenia/etiologia
19.
Reumatismo ; 71(2): 92-98, 2019 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-31309781

RESUMO

A 62-year-old man with a history of systemic sclerosis was admitted with diffuse alveolar hemorrhage and acute kidney injury without clinical data suggestive of glomerulonephritis. Laboratory tests showed anemia, leukocytosis with neutrophilia, thrombocytopenia, elevated serum creatinine and metabolic acidosis. Antinuclear antibodies were positive at a titer of 1/640 (speckled, 1/160; nucleolar, 1/320) while rheumatoid factor, anti Scl-70, anti-centromere, anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibodies were negative and serum complement levels were within normal range. During the following days, the patient developed multiple organ failure and, eventually, died. Lupus anticoagulant was revealed positive after the patient's death, suggesting a catastrophic antiphospholipid syndrome. Clinical data and autopsy were consistent with this diagnosis.


Assuntos
Síndrome Antifosfolipídica/etiologia , Escleroderma Sistêmico/complicações , Doença Catastrófica , Humanos , Masculino , Pessoa de Meia-Idade
20.
Zhonghua Nei Ke Za Zhi ; 58(7): 496-500, 2019 Jul 01.
Artigo em Chinês | MEDLINE | ID: mdl-31269565

RESUMO

Antiphospholipid antibodies (aPLs) are a group of autoantibodies that target phospholipids and/or phospholipid-binding proteins. aPLs are important serum markers of anti phospholipid syndrome and are also risk factors for thrombosis and pathological pregnancy. Standardization of aPLs detection is critical to its clinical application.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/diagnóstico , Trombose/imunologia , Anticorpos Anticardiolipina/sangue , Anticorpos Anticardiolipina/imunologia , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Biomarcadores/sangue , Consenso , Feminino , Humanos , Gravidez , Trombose/prevenção & controle , beta 2-Glicoproteína I
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