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Despite anticoagulation recommendations, patients may present with recurrent events. While medication adherence is always a concern, assessment of anticoagulation failure demands a systematic approach, taking into account the potential limitations of anticoagulants and a review of differential diagnoses for comorbidities. We illustrate our approach in a case presentation.
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INTRODUCTION: The aim of this study was to investigate the frequency of avascular necrosis (AVN) and its associations with clinical and laboratory parameters in patients with primary antiphospholipid antibody syndrome (pAPS). METHODS: This was a cross-sectional study of 66 patients with pAPS who fulfilled the Sidney criteria for APS. Epidemiological and clinical data, and data on antiphospholipid antibodies were collected. Diagnosis of AVN was based on studies of magnetic resonance (MRI) images of affected bones. RESULTS: AVN was found in three of the 66 (4.5%) enrolled patients. Comparison of patients with pAPS with and without AVN showed that demographic data, comorbidities, and antiphospholipid antibodies profile were similar in both groups. Compared to patients without AVN, those with AVN had thrombocytopenia more frequently (66.7 vs. 7.9%) and used more glucocorticoids (100 vs. 35%). CONCLUSION: AVN is rare in patients with pAPS and may be associated with thrombocytopenia and previous glucocorticoid use.
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Antiphospholipid antibody syndrome (APS) is a state of hypercoagulability secondary to an autoimmune disorder. It is associated with thrombotic events in venous and arterial vessels, obstetric complications characterized by recurrent fetal losses, and increased perinatal morbidity. APS is classified as primary, when not associated with other pathologies; or secondary, when associated with an underlying autoimmune disease with, solid tumor, or hematological disorder. Clinical findings include livedo reticularis, thrombocytopenia or hemolytic anemia, maternal morbidity, and recurrent thrombotic episodes and others. Laboratory tests show circulating antiphospholipid antibodies (aPLs); however, even in the presence of these antibodies, patients can be asymptomatic. Estimates predict that about 5% of the populations have circulating aPLs, but the incidence of APS is only five cases per 100,000 people, as diagnosis of this syndrome requires clinical and laboratory findings to be simultaneously present. In cases of secondary APS, or in acute cases with imminent risk of death (as in catastrophic APS), it may be necessary to reduce aPL serum levels using immunomodulators, immunosuppressants, or plasmapheresis, in order to treat the associated pathologies. In other situations, the use of immunotherapy is not indicated. In other patients heparin, aspirin or anticoagulants either alone or associated should be administered depending on each specific case.
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Abstract 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 yearswas 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 andinflammatory signaling pathways, to provide important advances in its treatment.
Resumo A Síndrome do anticorpo antifosfolípide (SAF) é uma doença sistêmica, autoimune e prótrombótica caracterizada por anticorpos antifosfolípides, trombose, aborto recorrente, complicações durante a gestação, e, ocasionalmente, trombocitopenia. O objetivo do presente estudo foi revisar a fisiopatologia da SAF e sua associação com a infertilidade feminina. Foi feita uma revisão bibliográfica dos últimos 20 anos nas bases de dados PubMed, Scielo e Bireme. A SAF pode estar associada à infertilidade primária, interferindo na decidualização endometrial e combaixas reservas ovarianas. Os anticorpos antifosfolípides também apresentam efeito negativo direto na placentação, se ligando ao trofoblasto e diminuindo sua capacidade de invasão, além de efeitos pró-inflamatórios, tais como ativação do sistema de complemento e recrutamento de neutrófilos, contribuindo para a insuficiência placentária, crescimento intrauterino restrito e perda fetal.Quanto a trombose, a SAF resulta em distúrbios trombóticos difusos, com uma desregulação do balanço homeostático. Conhecer a fisiopatologia da SAF, que apresenta associação importante com a infertilidade feminina, é essencial para novas abordagens terapêuticas, principalmente no que tange imunomodulação e os caminhos de ativação inflamatórios.
Assuntos
Humanos , Feminino , Gravidez , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/fisiopatologia , Infertilidade Feminina/complicações , Infertilidade Feminina/fisiopatologia , Aborto Habitual , Anticorpos Antifosfolipídeos/sangue , Pessoa de Meia-IdadeRESUMO
Antiphospholipid syndrome is a complex autoimmune disease, characterized by the presence of vascular thrombosis, obstetric, hematologic, cutaneous, and cardiac manifestations. Renal disease in patients with antiphospholipid syndrome was not recognized in the first descriptions of the disease, but later on, the renal manifestations of the syndrome have been investigated widely. Renal manifestations of antiphospholipid syndrome conform a wide spectrum of diverse renal syndromes. Hypertension is one of the most frequent, but less commonly recognized renal alteration. It can be difficult to control as its origin is renovascular. Renal vascular thrombosis can be arterial or venous. Other alterations are renal infarction and vascular thrombosis in arterial territories. Venous thrombosis can be present in primary and secondary antiphospholipid syndrome; it presents with worsening of previous proteinuria or de novo nephrotic syndrome, hypertension and renal failure. Antiphospholipid syndrome nephropathy is a vascular disease that affects glomerular tuft, interstitial vessels, and peritubular vessels; histopathology characterizes the renal lesions as acute or chronic, the classic finding is thrombotic microangiopathy, that leads to fibrosis, tubule thyroidization, focal cortical atrophy, and glomerular sclerosis. Antiphospholipid syndrome nephropathy can also complicate patients with systemic lupus erythematosus, and there is vast information supporting the worse renal prognosis in this group of patients with the classic histopathologic lesions. Treatment consists of anticoagulation, as for other thrombotic manifestations of antiphospholipid syndrome. There is some evidence of glomerulonephritis as an isolated lesion in patients with antiphospholipid syndrome. The most frequently reported glomerulonephritis is membranous; with some reports suggesting that immunosuppressive treatment may be effective. Patients with end stage renal disease commonly are positive for antiphospholipid antibodies, but it is not clear what is the role of aPL in this setting. Patients with vascular access may have complications in the presence of antibodies so that anticoagulation is recommended. Patients ongoing renal transplant with persistent antiphospholipid antibody positivity may have early and late graft failure.
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Síndrome Antifosfolipídica/complicações , Nefropatias/etiologia , Rim/patologia , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/fisiopatologia , Doenças Autoimunes/complicações , Doenças Autoimunes/fisiopatologia , Humanos , Hipertensão/etiologia , Falência Renal Crônica/etiologia , Lúpus Eritematoso Sistêmico/complicações , Síndrome Nefrótica/etiologia , Trombose/etiologiaRESUMO
The antiphospholipid antibody syndrome (APS) is defined by a state of hypercoagulability secondary to an autoimmune disorder. There are evidences that approximately 2.8-5.5% of cases of acute myocardial infarction (AMI) in young individuals are secondary to APS. In this case report, three coronary artery thromboses occurring within a short period are described. Initially, there was an ST-segment elevation (STEMI) in the presence of coronary artery disease (CAD), with the vessel being treated with stent implantation. Thereafter, a subacute stent thrombosis occurred (high thrombotic load in almost all coronary arteries), which was treated with implantation of two stents. Subsequently, there was a new infarction owing to a new thrombosis in the native coronary artery. The treatment of APS in patients who developed thrombotic events is full anticoagulation from the initial stages maintained throughout life. The standard anticoagulant therapy is administration of vitamin K antagonists, such as warfarin.
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Antiphospholipid antibody syndrome is a heterogeneous clinical and biochemical entity. We present the case of a young male with history of venous thromboembolism. This time he presents because of chest ischemic pain associated with ST segment elevation. He was taken to the cath lab to perform a primary percutaneous coronary intervention and a total occlusion of the left anterior descending artery was noted. Successful thrombus aspiration was performed. No stent was deployed. He was taken to the cath lab for a second look angiography and no atherosclerotic lesions were observed, which was confirmed by intravascular ultrasound.
El síndrome de anticuerpos antifosfolípidos es una situación clínica y bioquímica heterogénea. Presentamos el caso de un varón joven con antecedente de tromboembolia venosa que se presentó en esta ocasión por dolor precordial, con elevación del ST en el electrocardiograma. Fue llevado a sala de angiografía para realizar angioplastia primaria y se observó una oclu- sión total ostial de la descendente anterior. Se realizó aspiración manual del trombo. No se realizó angioplastia con balón ni stent. En la angiografía de control a las 48 horas se observó ausencia de trombo y de placas aterosclerosas, lo cual se co- rroboró mediante ultrasonido intracoronario.
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Síndrome Antifosfolipídica/complicações , Trombose Coronária/etiologia , Infarto do Miocárdio com Supradesnível do Segmento ST/etiologia , Adulto , Dor no Peito/etiologia , Trombose Coronária/complicações , Trombose Coronária/terapia , Humanos , Masculino , Intervenção Coronária Percutânea/métodos , Infarto do Miocárdio com Supradesnível do Segmento ST/terapiaRESUMO
OBJECTIVE: The association of antiphospholipid antibody syndrome (APS) and hypercoagulability is well known. Arterial compromise leading to ischemia of organs and/or limbs in patients with APS is uncommon, frequently unrecognized, and rarely described. We evaluated our institutional experience. METHODS: Retrospective review was conducted. From August 2007 to September 2016, 807 patients with diagnosis of APS were managed in our Institution. Patients with primary and secondary APS who required interventions were examined. Demographics, comorbidities, manifestations, procedures, complications, and other factors affecting outcomes were recorded. RESULTS: Fourteen patients (mean age 35 years old, standard deviation ±14) were evaluated and treated by our service. Six (43%) of them had primary APS and 8 (57%) had secondary APS; 11 (79%) were female. Two (14%) experienced distal aorta and iliac arteries involvement, 3 (21%) visceral vessels disease, 2 (14%) in upper and 7 (50%) in the lower extremity vasculatures. Thirteen (93%) patients underwent direct open revascularization and 1 with hand ischemia (Raynaud disease) underwent sympathectomy. During the mean follow-up period of 48 months, reinterventions included a revision of the proximal anastomosis of an aortobifemoral bypass graft, 1 (7%) abdominal exploration for bleeding, 1 (7%) graft thrombectomy, and 4 (29%) amputations (2 below the knee, 1 above the knee, and 1 transmetatarsal). One (7%) death occurred secondary to sepsis in a patient who had acute mesenteric ischemia. Significant differences in clinical manifestations and outcomes were not observed among patients with primary and secondary APS. All patients remained on systemic anticoagulation. CONCLUSION: APS is a prothrombotic disorder that may lead to arterial involvement with less frequency than the venous circulation but has significant morbidity and limb loss rate. Arterial reconstruction seems feasible in an attempt to salvage organs and limbs; however, research is necessary to establish the optimal anticoagulation regime and long-term management following surgical interventions.
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Síndrome Antifosfolipídica/complicações , Isquemia/cirurgia , Doença Arterial Periférica/cirurgia , Trombofilia/etiologia , Procedimentos Cirúrgicos Vasculares , Adulto , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/mortalidade , Aortografia/métodos , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Isquemia/diagnóstico por imagem , Isquemia/etiologia , Isquemia/mortalidade , Masculino , Pessoa de Meia-Idade , Doença Arterial Periférica/diagnóstico por imagem , Doença Arterial Periférica/etiologia , Doença Arterial Periférica/mortalidade , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Reoperação , Estudos Retrospectivos , Fatores de Risco , Trombofilia/diagnóstico , Trombofilia/tratamento farmacológico , Trombofilia/mortalidade , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/mortalidade , Adulto JovemRESUMO
The objective of the study is to study the positivity of Coombs test or direct antiglobulin test (DAT) in systemic lupus erythematosus (SLE) patients and its relationship with disease's clinical and serological profile. Retrospective study of 373 SLE patients seen at single Rheumatology Unit. Epidemiological data (age, gender, age at disease onset, auto declared ethnic background and tobacco use), clinical (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia and arthritis), and serological profile (anti ds DNA, anti Ro/SS-A; anti La/SS-B, Anti RNP, Anti Sm, aCl (anticardiolipin) IgG, aCl Ig M, LA or lupus anticoagulant, rheumatoid factor and direct Coombs) were collected. Patients with a positive DAT were compared with the negatives. DAT was positive in 12.8% of patients and 54.3% of them had hemolytic anemia. In univariate analysis, a positive DAT was associated with hemolytic anemia (p < 0.0001), secondary antiphospholipid antibody syndrome (p = 0.02), anticardiolipin IgG (p < 0.0001), lupus anticoagulant (p = 0.05), positive VDRL (p = 0.004), anti-RNP (p < 0.0001), anti-Sm (p = 0.002), and anti-La (p = 0.02). Logistic regression revealed that hemolytic anemia, anti-RNP and anti-LA were independently associated with positive DAT. DAT was positive in 12.8% of SLE studied sample and 54.3% of them had hemolytic anemia. This test was independently associated with hemolytic anemia, anti-RNP and anti-La antibodies.
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Anemia Hemolítica Autoimune/diagnóstico , Anticorpos Antinucleares/sangue , Síndrome Antifosfolipídica/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Trombocitopenia/diagnóstico , Adulto , Feminino , Humanos , Modelos Logísticos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Pregnancy in women with autoimmune rheumatic diseases is associated with several maternal and fetal complications. The development of clinical practice guidelines with the best available scientific evidence may help standardize the care of these patients. OBJECTIVES: To provide recommendations regarding prenatal care, treatment, and a more effective monitoring of pregnancy in women with lupus erythematosus, rheumatoid arthritis (RA) and antiphospholipid syndrome (APS). METHODOLOGY: Nominal panels were formed for consensus, systematic search of information, development of clinical questions, processing and staging of recommendations, internal validation by peers and external validation of the final document. The quality criteria of the AGREE II instrument were followed. RESULTS: The panels answered 37 questions related to maternal and fetal care in lupus erythematosus, RA and APS, as well as for use of antirheumatic drugs during pregnancy and lactation. The recommendations were discussed and integrated into a final manuscript. Finally, the corresponding algorithms were developed. In this second part, the recommendations for pregnant women with RA, APS and the use of antirheumatic drugs during pregnancy and lactation are presented. CONCLUSIONS: We believe that the Mexican clinical practice guidelines for the management of pregnancy in women with RA and APS integrate the best available evidence for the treatment and follow-up of patients with these conditions.
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Síndrome Antifosfolipídica/terapia , Artrite Reumatoide/terapia , Lúpus Eritematoso Sistêmico/terapia , Complicações na Gravidez/terapia , Cuidado Pré-Natal/métodos , Assistência ao Convalescente/métodos , Síndrome Antifosfolipídica/diagnóstico , Antirreumáticos/uso terapêutico , Artrite Reumatoide/diagnóstico , Tomada de Decisão Clínica , Técnicas de Apoio para a Decisão , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , México , Gravidez , Complicações na Gravidez/diagnósticoRESUMO
Relato de caso de paciente de 38 anos, feminina, com lúpus eritematoso sistêmico (LES) que apresentou evento tromboembólico arterial agudo em membro inferior direito. A investigação evidenciou a presença de anticorpos antifosfolípides e vegetação asséptica em válvula mitral, endocardite de Libman-Sacks (eLS). São discutidas as possíveis causas de eventos tromboembólicos arteriais no LES, com ênfase nas recomendações atuais para diagnóstico e tratamento da eLS.
Case report of a 38-year-old female patient with systemic lupus erythematosus (SLE) who presented an acute arterial thromboembolic event in the right lower limb. Investigation showed the presence of antiphospholipid antibodies and sterile vegetation in the mitral valve, Libman-Sacks endocarditis (LSE). Possible causes of thromboembolic events in SLE are discussed, with emphasis on current recommendations for diagnosis and treatment of LSE.
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Adulto , Feminino , Humanos , Anticorpos Antifosfolipídeos/imunologia , Endocardite/etiologia , Endocardite/imunologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/imunologia , Trombose/etiologia , Trombose/imunologiaRESUMO
OBJETIVO: Avaliar a eficácia da intervenção nutricional na redução do excesso de peso (EP), em pacientes com síndrome do anticorpo antifosfolípide (SAF). MÉTODO: Incluídos 40 pacientes, acima de 18 anos, com diagnóstico de SAF primária ou secundária, acompanhados no Serviço de Reumatologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP) e recrutados no período de outubro de 2005 a fevereiro de 2006. Foram coletados dados demográficos e realizados a revisão de prontuários, a mensuração de peso e da altura, o cálculo do índice de massa corpórea (IMC) atual e a adequação da dieta pelo IMC ideal. Foi realizada avaliação na primeira consulta e após intervalo mínimo de dois meses. RESULTADOS: A média de idade dos pacientes foi de 41 anos, sendo 93 por cento de mulheres. Os pacientes com SAF primária perfaziam 25 por cento, com média de dois anos da doença. A trombose venosa ocorreu em 63 por cento, arterial em 48 por cento e manifestações obstétricas em 27 por cento. Na primeira consulta, 68 por cento apresentavam EP, 27 por cento eram eutróficos e 5 por cento estavam com baixo peso (BP). Após três meses de intervenção, os eutróficos mantiveram o peso e os de BP tornaram-se eutróficos, segundo o IMC. Interessantemente, entre os pacientes com EP (n = 27), 82 por cento emagreceram, 14 por cento engordaram e 4 por cento se mantiveram. Especificamente, 11 pacientes apresentaram 1 por cento a 3 por cento de perda ponderal de peso, oito perderam de 4 por cento a 7 por cento, dois reduziram 8 por cento a 9 por cento e um reduziu 13,6 por cento com o acompanhamento nutricional. CONCLUSÃO: Foi demonstrado no presente estudo que a intervenção nutricional conseguiu atingir metas para redução de peso, possibilitando diminuição no risco trombótico num curto período, sendo, portanto, uma modalidade terapêutica inicial e de eleição para corrigir o EP em pacientes com SAF.
OBJECTIVE: To evaluate the efficacy of the nutricional intervention in the loss of the weight excess (WE), in patients with the antiphospholipid antibody syndrome (APS). METHODS: Forty patients older than 18 years-old were included, with a diagnosis of either primary or secondary APS, followed at the the Rheumatology Outpatient Clinic from HCFM-USP and were recruited between october/2005 and february/06. Demographic data and patient records were reviewed. Body weight, height and current Body Mass Index (BMI) were recorded as well as diet adjustment according to ideal BMI. Patients were subjected to a first medical and nutritional evaluation and subsequently at least 2 months after baseline. RESULTS: The mean age was 41 years old and 93 percent of the patients were female. The patients with primary APS were 25 percent, the mean disease duration was 2 years. Venous thrombosis occurred in 63 percent, arterial in 48 percent and obstetric events in 27 percent. In the first consultation 68 percent presented WE, 27 percent were euthrophic and 5 percent were of low weight (LW). After three months of intervention, the eutrophics maintained body weight while those with LW became eutrophic. Among the patients with WE (n=27), 82 percent lost weight, 14 percent had gained weight and 4 percent kept their weight. Specifically, 11 patients had-3 percent of weight loss, 8 lost 4-7 percent, 2 lost 8-9 percent, and 1 patient lost 13.6 percent while under nutritional counseling. CONCLUSION: The data demonstrate that nutritional intervention help achieving reduction in body weight. This may lead to reduction of the thrombotic risk in a short period of time. Nutritional intervention may thus be a valuable initial therapeutic approach to adjust body weight in patients with APS.
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Humanos , Masculino , Feminino , Síndrome Antifosfolipídica , Educação Alimentar e Nutricional , Doenças Metabólicas , Obesidade , Sobrepeso , Redução de PesoRESUMO
Se presentan dos casos de gestantes con diagnóstico previo de síndrome de anticuerpos antifosfolipídicos (SAF) con historias de pérdidas de embarazos anteriores por diversos motivos, que tuvieron un seguimiento durante todo el embarazo y se aplicaron tratamientos distintos de acuerdo a la situación clínica. Se obtuvieron resultados perinatales diferentes. Se hacen comentarios con relación a la terapéutica utilizada y la repercusión de esta enfermedad en el embarazo, con comentarios finales basados en nuestra experiencia.
Two cases of pregnant women with previous diagnosis of antiphospholipid antibody syndrome that had lost previous pregnancies for different reasons are presented. They were followed-up during the whole gestation and diverse treatments were applied according to the clinical situation. Various perinatal results were obtained. Comments are made on the therapeutics used and the impact of this disease on pregnancy. Finally, observations based on our experience are exposed.