RESUMO
Background/Purpose Long-term anticoagulation is the standard treatment for thrombotic antiphospholipid syndrome (APS). However, in daily practice, the question of withdrawing anticoagulation may arise, without any evidence-based recommendations. This study aimed to assess outcomes in APS patients after anticoagulation withdrawal. Methods Thrombotic APS patients followed in our centre, whose anticoagulation was withdrawn after APS diagnosis, were retrospectively selected, and were match-controlled with patients under anticoagulation, based on sex, age, APS clinical phenotype and disease duration. Results Thirty cases with anticoagulation withdrawal were included. Median follow-up was 51 months (12-124). The risk of thrombotic relapse was higher in cases compared to controls (7.3% versus 1.5% patient-year ( p = 0.01); hazard ratio 4.8; 95% confidence interval (1.4-16.7)). Male gender, anti-ß2GP1 and triple positivity at inclusion were predictive factors for thrombotic relapse. Conversely, aspirin prescription was a protective factor against relapses. Persistence of LA, anti-ß2GP1 and triple positivity over time were associated with a higher risk of thrombosis and aPL disappearance with a lower risk. Conclusion In our study, anticoagulation withdrawal was associated with an increased risk of thrombotic relapse. Our findings emphasize the influence of anti-ß2GP1 and triple positivity persistence over time on the risk of relapse and the benefit of aspirin prescription when anticoagulation has been withdrawn.
Assuntos
Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Trombose/complicações , Trombose/tratamento farmacológico , Adulto , Anticorpos Antifosfolipídeos/imunologia , Anticoagulantes/uso terapêutico , Aspirina/uso terapêutico , Coagulação Sanguínea/efeitos dos fármacos , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Recidiva , Estudos Retrospectivos , Fatores de Risco , Adulto JovemRESUMO
Purpose The purpose of this study was to evaluate the safety of antithrombotic treatments prescribed during pregnancy in patients with antiphospholipid syndrome (APS). Methods This international, multicenter study included two cohorts of patients: a retrospective French cohort and a prospective US cohort (PROMISSE study). Inclusion criteria were (1) APS (Sydney criteria), (2) live pregnancy at 12 weeks of gestation (WG) with (3) follow-up data until six weeks post-partum. According to APS standard of care, patients were treated with aspirin and/or low-molecular weight heparin (LMWH) at prophylactic (pure obstetric APS) or therapeutic doses (history of thrombosis). Major bleeding was defined as abnormal blood loss during the pregnancy and/or post-partum period requiring intervention for hemostasis or transfusion, or during the peripartum period greater than 500 mL and/or requiring surgery or transfusion. Other bleeding events were classified as minor. Results Two hundred and sixty-four pregnancies (87 prospectively collected) in 204 patients were included (46% with history of thrombosis, 23% with associated systemic lupus). During pregnancy, treatment included LMWH ( n = 253; 96%) or low-dose aspirin ( n = 223; 84%), and 215 (81%) patients received both therapies. The live birth rate was 89% and 82% in the retrospective and prospective cohorts, respectively. Adverse pregnancy outcomes occurred in 28% of the retrospective cohort and in 40% of the prospective cohort. No maternal death was observed in either cohort. A combined total of 45 hemorrhagic events (25%) occurred in the retrospective cohort, but major bleeding was reported in only six pregnancies (3%). Neither heparin nor aspirin alone nor combined therapy increased the risk of hemorrhage. We also did not observe an increased rate of bleeding in the case of a short interval between last LMWH (less than 24 hours) or aspirin (less than five days) doses and delivery. Only emergency Caesarean section was significantly associated with an increased risk of bleeding (odds ratio (OR) 5.03 (1.41-17.96); p=.016). In the prospective cohort, only one minor bleeding event was reported (vaginal bleeding). Conclusion Our findings support the safety of antithrombotic therapy with aspirin and/or LMWH during pregnancy in high-risk women with APS, and highlight the need for better treatments to improve pregnancy outcomes in APS. PROMISSE Study ClinicalTrials.gov identifier: NCT00198068.
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Anticoagulantes/efeitos adversos , Síndrome Antifosfolipídica/tratamento farmacológico , Aspirina/efeitos adversos , Fibrinolíticos/efeitos adversos , Heparina de Baixo Peso Molecular/efeitos adversos , Inibidores da Agregação Plaquetária/efeitos adversos , Hemorragia Pós-Parto/induzido quimicamente , Adulto , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/diagnóstico , Perda Sanguínea Cirúrgica/prevenção & controle , Transfusão de Sangue , Cesárea/efeitos adversos , Quimioterapia Combinada , Feminino , França , Humanos , Hemorragia Pós-Operatória/induzido quimicamente , Hemorragia Pós-Operatória/terapia , Hemorragia Pós-Parto/diagnóstico , Hemorragia Pós-Parto/terapia , Gravidez , Estudos Prospectivos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Estados UnidosRESUMO
Introduction The long-term risk of first thrombosis and benefit of prophylaxis in antiphospholipid antibody (aPL) carriers without history of thrombosis or obstetrical morbidity is poorly known. This study aimed to evaluate the long-term rate and risk factors associated with a first thrombosis in those patients. Patients and methods After a prior study ended in December 2005 and was already published, we extended the follow-up period of our cohort of aPL carriers. Results Ninety-eight of the 103 patients of the previous study were included. The annual first thrombosis rate was 2.3% per patient-year during a median of 13 years (6-17). None of the baseline characteristics was predictive of risk of first thrombosis, but persistent aPL over time were associated with an increased risk. The stronger association was found in triple aPL-positive carriers: OR 3.38 (95% CI: 1.24-9.22). Of note, conversely to our previous findings, no benefit of aspirin prophylaxis was observed. Conclusion The risk of first thrombosis in aPL carriers without history of thrombosis or obstetrical morbidity was significant, persisted linearly over time and was associated with persistent aPL. This risk was especially increased in triple aPL-positive carriers, in whom a close follow-up seems to be necessary. Nevertheless, the benefit of aspirin prophylaxis remained unclear.
Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Trombose/etiologia , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Aspirina/administração & dosagem , Biomarcadores/sangue , Distribuição de Qui-Quadrado , Intervalo Livre de Doença , Feminino , Fibrinolíticos/administração & dosagem , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Trombose/sangue , Trombose/diagnóstico , Trombose/prevenção & controle , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Patients with chronic illnesses, especially rare autoimmune and/or systemic diseases associated with significant diagnostic uncertainty, have a representation of their illness and a sometimes prolonged hospitalization experience that can be traumatic and anxiety-provoking. OBJECTIVE: The aim of this study was to evaluate the impact of a non-medicinal medical hypnosis intervention in reducing the stress state and improving the experience of patients hospitalized in an internal medicine department. METHODS: We conducted a prospective study of 24 patients hospitalized in the Internal Medicine Department of Lille University Hospital in 2023. Twelve patients received a non-drug medical hypnosis intervention known as the "place of safety" (case group) and were compared with 12 patients who did not (control group). Stress was assessed by the STAI questionnaire and hospitalization experience by a satisfaction questionnaire. RESULTS: The 24 patients, 13 of whom were women, had a mean age of 55±17 years at inclusion. On admission to hospital, the median STAI-ETAT between the two groups was 43.5 (38.0; 56.6) in the case group versus 42.0 (37.0; 48.5) in the control group (P=0.45). In the case group, the median STAI-ETAT questionnaire taken immediately after the hypnosis session was significantly lower than at the start of hospitalization (30.0 [25.5; 36.5] vs. 43.5 [38.0; 56.5] P=0.003), indicating a significant reduction in stress. At the end of hospitalization, there was also a significant persistence of the median significant reduction between cases and controls (29.5 [26.5; 35.0] for cases vs. 41.5 [33.5; 45.5] for controls P=0.002). Experience of hospitalization was better in the case group (median 5.0 [4.5; 5.0] vs. 4.0 [4.0; 4.5], P=0.016). CONCLUSION: This study suggests that medical hypnosis is a promising non-medicinal supportive intervention for reducing perceived stress and improving the experience of stress in patients hospitalized on an internal medicine ward.
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Hospitalização , Hipnose , Medicina Interna , Estresse Psicológico , Humanos , Feminino , Hipnose/métodos , Medicina Interna/estatística & dados numéricos , Medicina Interna/métodos , Masculino , Pessoa de Meia-Idade , Hospitalização/estatística & dados numéricos , Estresse Psicológico/terapia , Estresse Psicológico/epidemiologia , Estresse Psicológico/etiologia , Estresse Psicológico/psicologia , Idoso , Adulto , Estudos Prospectivos , Inquéritos e Questionários , Departamentos Hospitalares/estatística & dados numéricos , Satisfação do Paciente/estatística & dados numéricosRESUMO
INTRODUCTION: Coronaritis is a rare but serious complication of giant-cell arteritis (GCA), with an estimated prevalence of less than 1%, however difficult to establish, and of early onset. METHODS: We describe 2 cases of GCA presenting with coronaritis and present a review of the literature on this complication. RESULTS: The first patient presented with stable angina on common trunk coronaritis with ostial stenosis. Corticosteroid combined with tocilizumab from the outset resulted in improvement. Angioplasty was performed at 6months with good outcome. The second patient presented with asymptomatic tritruncular ostial coronaritis. Corticosteroid allowed clinic-biological improvement of GCA. Two years later, he presented relapse with an acute coronary syndrome, with favorable evolution after angioplasty, increase of corticosteroids and addition of tocilizumab. CONCLUSION: Patients presented were successfully treated with corticosteroids combined with tocilizumab and angioplasty of their coronary stenoses. Efficacy of tocilizumab in GCA has not been evaluated especially on coronaritis due to the rarity of this complication. Our experience and the cases reported in the literature suggest good results of angioplasty in this indication. Studies with long-term follow-up will be necessary to evaluate the risk of restenosis.
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Arterite de Células Gigantes , Humanos , Masculino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/terapia , Angioplastia , Corticosteroides/uso terapêuticoRESUMO
Antiphospholipid syndrome (APS) is a chronic autoimmune disease involving vascular thrombosis and/or obstetric morbidity and persistent antibodies to phospholipids or certain phospholipid-associated proteins. It is a rare condition in adults and even rarer in children. The diagnosis of APS can be facilitated by the use of classification criteria based on a combination of clinical and biological features. APS may be rapidly progressive with multiple, often synchronous thromboses, resulting in life-threatening multiple organ failure. This form is known as "catastrophic antiphospholipid syndrome" (CAPS). It may be primary or associated with systemic lupus erythematosus (associated APS) and in very rare cases with other systemic autoimmune diseases. General practitioners and paediatricians may encounter APS in patients with one or more vascular thromboses. Because APS is so rare and difficult to diagnosis (risk of overdiagnosis) any suspected case should be confirmed rapidly and sometimes urgently by an APS specialist. First-line treatment of thrombotic events in APS includes heparin followed by long-term anticoagulation with a VKA, usually warfarin. Except in the specific case of stroke, anticoagulants should be started as early as possible. Any temporary discontinuation of anticoagulants is associated with a high risk of thrombosis in APS. A reference/competence centre specialised in autoimmune diseases must be urgently consulted for the therapeutic management of CAPS.
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Síndrome Antifosfolipídica , Doenças Autoimunes , Lúpus Eritematoso Sistêmico , Trombose , Gravidez , Feminino , Humanos , Adulto , Criança , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Anticorpos Antifosfolipídeos , Anticoagulantes/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Trombose/diagnóstico , Trombose/epidemiologia , Trombose/etiologia , Doenças Autoimunes/complicaçõesRESUMO
Hypercholesterolemia refers to dyslipidemia with an increased circulating cholesterol levels. This is the most common dyslipidemia and is associated with an increased risk of developing atheromatous cardiovascular diseases. One of the major challenges in primary prevention is to define the threshold for therapeutic intervention that allow to obtain a significant clinical benefit without unnecessarily expose the patient to potential side effects of lipid-lowering treatments. It is also important to recall to screen patient for heterozygous familial hypercholesterolemia, a common genetic disease of lipid metabolism responsible for particularly severe and early coronary disease. In this article, the issues of hypercholesterolemia screening, the definition of therapeutic targets and expected benefits as well as the modalities of therapeutic management (by also addressing the problem of statin intolerance) will be addressed.
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Doenças Cardiovasculares , Inibidores de Hidroximetilglutaril-CoA Redutases , Hipercolesterolemia , Hiperlipoproteinemia Tipo II , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , LDL-Colesterol , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Hipercolesterolemia/diagnóstico , Hipercolesterolemia/epidemiologia , Hipercolesterolemia/terapia , Hiperlipoproteinemia Tipo II/complicações , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/epidemiologiaRESUMO
BACKGROUND: As lack of awareness of rare diseases (RDs) among healthcare professionals results in delayed diagnoses, there is a need for a more efficient approach to RD training during academic education. We designed an experimental workshop that used role-play simulation with patient educators and focused on teaching "red flags" that should raise the suspicion of an RD when faced with a patient with frequently encountered symptoms. Our objective was to report our experience, and to assess the improvement in learners' knowledge and the satisfaction levels of the participants. RESULTS: The workshop consisted of 2 simulated consultations that both started with the same frequent symptom (Raynaud phenomenon, RP) but led to different diagnoses: a frequent condition (idiopathic RP) and an RD (systemic sclerosis, SSc). In the second simulated consultation, the role of the patient was played by a patient educator with SSc. By juxtaposing 2 seemingly similar situations, the training particularly highlighted the elements that help differentiate SSc from idiopathic RP. When answering a clinical case exam about RP and SSc, students that had participated in the workshop had a higher mean mark than those who had not (14 ± 3.7 vs 9.6 ± 5.5 points out of 20, p = 0.001). Participants mostly felt "very satisfied" with this training (94%), and "more comfortable" about managing idiopathic RP and SSc (100%). They considered the workshop "not very stressful" and "very formative" (both 71%). When asked about the strengths of this training, they mentioned the benefits of being put in an immersive situation, allowing a better acquisition of practical skills and a more interactive exchange with teachers, as well as the confrontation with a real patient, leading to a better retention of semiological findings and associating a relational component with this experience. CONCLUSIONS: Through the use of innovative educational methods, such as role-play simulation and patient educators, and by focusing on teaching "red flags", our workshop successfully improved RP and SSc learning in a way that satisfied students. By modifying the workshop's scenarios, its template can readily be applied to other clinical situations, making it an interesting tool to teach other RDs.
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Doença de Raynaud , Escleroderma Sistêmico , Humanos , Doenças Raras , Escleroderma Sistêmico/diagnósticoRESUMO
PURPOSE: Management of giant cell arteritis (GCA, Horton's disease) involves many uncertainties. This work was undertaken to establish French recommendations for GCA management. METHODS: Recommendations were developed by a multidisciplinary panel of 33 physicians, members of the French Study Group for Large Vessel Vasculitis (Groupe d'étude français des artérites des gros vaisseaux [GEFA]). The topics to be addressed, selected from proposals by group members, were assigned to subgroups to summarize the available literature and draft recommendations. Following an iterative consensus-seeking process that yielded consensus recommendations, the degree of agreement among panel members was evaluated with a 5-point Likert scale. A recommendation was approved when ≥ 80% of the voters agreed or strongly agreed. RESULTS: The 15 retained topics resulted in 31 consensus recommendations focusing on GCA nomenclature and classification, the role of temporal artery biopsy and medical imaging in the diagnosis, indications and search modalities for involvement of the aorta and its branches, the glucocorticoid regimen to prescribe, treatment of complicated GCA, indications for use of immunosuppressants or targeted biologic therapies, adjunctive treatment measures, and management of relapse and recurrence. CONCLUSIONS: The recommendations, which will be updated regularly, are intended to guide and harmonize the standards of GCA management.
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Arterite de Células Gigantes/terapia , Algoritmos , Membro de Comitê , Consenso , Conferências de Consenso como Assunto , Prova Pericial , França , Arterite de Células Gigantes/classificação , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/patologia , Humanos , Medicina Interna/organização & administração , Sociedades Médicas/organização & administraçãoRESUMO
Through recreational activities of a body-expression group on an out-patient basis with a group of teenage psychotics, an attempt to elaborate the therapeutic effects, using the body, including certain dance techniques under defined and described conditions, is suggested. Where, at the outset, seems not to have been respected sexually, or on the other hand, to have been put into highly conflict--causing situations, when childhood has been marked by great bodily traumatism, when communication can no longer be carried out by words, this kind of physical approach can, by going beyond the psychotic defense mechanism, lead to a genuine narcissistic respect with all its positive effects concerning self-concept and in helping the person relate to others.