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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 Methods Thrombotic APS patients followed in our centre, whose anticoagulation was withdrawn after APS diagnosis, were retrospectively selected, and were match-controlled with patients under anticoagulation, based on sex, age, APS clinical phenotype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persistence over time on the risk of relapse and the benefit of aspirin prescription when anticoagulation has been withdrawn.
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Anticoagulantes/administración & dosificación , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/tratamiento farmacológico , Trombosis/complicaciones , Trombosis/tratamiento farmacológico , Adulto , Anticuerpos Antifosfolípidos/inmunología , Anticoagulantes/uso terapéutico , Aspirina/uso terapéutico , Coagulación Sanguínea/efectos de los fármacos , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Adulto JovenRESUMEN
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.
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Anticuerpos Antifosfolípidos/sangre , Síndrome Antifosfolípido/sangre , Trombosis/etiología , Adulto , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/tratamiento farmacológico , Aspirina/administración & dosificación , Biomarcadores/sangre , Distribución de Chi-Cuadrado , Supervivencia sin Enfermedad , Femenino , Fibrinolíticos/administración & dosificación , Estudios de Seguimiento , Humanos , Estimación de Kaplan-Meier , Modelos Lineales , Masculino , Persona de Mediana Edad , Análisis Multivariante , Oportunidad Relativa , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Trombosis/sangre , Trombosis/diagnóstico , Trombosis/prevención & control , Factores de Tiempo , Resultado del TratamientoRESUMEN
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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Hospitalización , Hipnosis , Medicina Interna , Estrés Psicológico , Humanos , Femenino , Hipnosis/métodos , Medicina Interna/estadística & datos numéricos , Medicina Interna/métodos , Masculino , Persona de Mediana Edad , Hospitalización/estadística & datos numéricos , Estrés Psicológico/terapia , Estrés Psicológico/epidemiología , Estrés Psicológico/etiología , Estrés Psicológico/psicología , Anciano , Adulto , Estudios Prospectivos , Encuestas y Cuestionarios , Departamentos de Hospitales/estadística & datos numéricos , Satisfacción del Paciente/estadística & datos numéricosRESUMEN
INTRODUCTION: Several studies suggest the relevance of healthcare simulation to prepare future doctors to deliver bad news. A such, we designed a role-play workshop to train first-year residents enrolled in Lille University School of Medicine to break bad news. The objective of this work is to report on our experience of this training and to assess its educational value through its capacity to satisfy residents' expectations, to induce a feeling of ease towards bad news disclosure, and to change trainees' preconceptions regarding these situations. METHODS: The training consisted of a 45-minute heuristic reflective activity, aimed at identifying residents' preconceptions regarding bad news disclosure, followed by 4 30-min role-plays in which they played the parts of the physician, the patient and/or their relatives. Trainees were asked to answer 2 questionnaires (pre- and post-training), exploring previous experiences, preconceived ideas regarding bad news disclosure and workshop satisfaction. RESULTS: Almost all residents felt very satisfied with the workshop, which they regarded as formative (91%) and not too stressful (89%). The majority felt "more capable" (53% vs. 83%) and "more comfortable" (27% vs. 62%) to deliver bad news, especially regarding "finding the right words" (12% vs. 22%). Trainees tended to overestimate their skills before the workshop and lowered their assessment of their performance after attending the training, especially when they played the role of a patient in the simulation. CONCLUSION: Healthcare role-play seems an interesting technique for training to breaking bad news. Placing residents in the role of patients or relatives is an active approach that encourages reflexivity.
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Internado y Residencia , Relaciones Médico-Paciente , Humanos , Revelación de la Verdad , Universidades , EscolaridadRESUMEN
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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Arteritis de Células Gigantes , Humanos , Masculino , Arteritis de Células Gigantes/complicaciones , Arteritis de Células Gigantes/diagnóstico , Arteritis de Células Gigantes/terapia , Angioplastia , Corticoesteroides/uso terapéuticoRESUMEN
OBJECTIVE: Longer-term effects of prolonged selective interleukin-1ß blockade with canakinumab were evaluated in the largest cohort of cryopyrin-associated periodic syndrome (CAPS) patients studied to date. METHODS: Adult and paediatric CAPS patients (n=166, including canakinumab-naive and pretreated patients from previous studies) received canakinumab subcutaneously 150 mg or 2 mg/kg (≤40 kg) every 8 weeks for up to 2 years. Response and relapse was assessed using scores for disease activity, skin rash and C-reactive protein (CRP) and/or serum amyloid A (SAA) levels. RESULTS: Complete response was achieved in 85 of 109 canakinumab-naive patients (78%; 79/85 patients within 8 days, and five patients between days 10 and 21). Of 141 patients with an available relapse assessment, 90% did not relapse, their CRP/SAA levels normalised (<10 mg/l) by day 8, and remained in the normal range thereafter. Median treatment duration was 414 days (29-687 days). Upward adjustments of dose or frequency were needed in 24.1% patients; mostly children and those with severe CAPS. Predominant adverse events (AE) were infections (65.7%) of mostly mild-to-moderate severity. Serious AE reported in 18 patients (10.8%) were mainly infections and were responsive to standard treatment. The majority of patients (92%) reported having no injection-site reactions and only 8% patients reported mild-to-moderate reactions. Patients receiving vaccination (15%) showed normal immune response. CONCLUSIONS: Subcutaneous canakinumab 150 mg every 8 weeks was well tolerated and provided substantial disease control in children and adults across all CAPS phenotypes. Higher canakinumab doses in younger patients and more severe CAPS disease were efficacious in achieving complete responses without evidence of increased AE. TRIAL REGISTRATION NUMBER: NCT00685373 (clinicaltrials.gov).
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Anticuerpos Monoclonales/administración & dosificación , Síndromes Periódicos Asociados a Criopirina/tratamiento farmacológico , Interleucina-1beta/antagonistas & inhibidores , Adolescente , Adulto , Anciano , Anticuerpos Monoclonales/efectos adversos , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales Humanizados , Peso Corporal , Proteína C-Reactiva/metabolismo , Niño , Preescolar , Síndromes Periódicos Asociados a Criopirina/inmunología , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Humanos , Inyecciones Subcutáneas , Persona de Mediana Edad , Fenotipo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Adulto JovenRESUMEN
INTRODUCTION: Hereditary angioedema (HAE) is characterized by recurrent attacks of swelling of various locations and severity. An impaired quality of life of patients with HAE has been reported by several studies. We aimed at examining the overall impact of the disease in patients followed for type I HAE, particularly its impact on daily life activities, emotions and quality of life. METHODS: A questionnaire was distributed to patients consulting for type I HAE, collecting demographics, disease characteristics, impact on professional life, Hospital Anxiety and Depression score (HAD), SF-36 score and the McMaster Toronto Arthritis Patient Preference Disability Questionnaire (MACTAR). RESULTS: The 33 patients included reported an average of 5.17 attacks over the last year. Stress was the main trigger A long-term treatment was reported by 58% of patients, 72% received specific treatment in the event of a serious attack. Sick days were reported by 33% of patients during their studies, and by 34% during work. One patient suffered from depressive symptoms and ten from anxious symptoms, according to the HAD score. The areas most impacted on the SF-36 score were general health and vitality. The mean score for MACTAR was low. CONCLUSION: HAE still has a significant impact on the daily and emotional lives of patients, despite the availability of prophylactic and crisis treatments.
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Angioedemas Hereditarios , Calidad de Vida , Angioedemas Hereditarios/diagnóstico , Angioedemas Hereditarios/epidemiología , Ansiedad/epidemiología , Ansiedad/etiología , Emociones , Humanos , Encuestas y CuestionariosRESUMEN
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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Enfermedad de Raynaud , Esclerodermia Sistémica , Humanos , Enfermedades Raras , Esclerodermia Sistémica/diagnósticoRESUMEN
INTRODUCTION: There are a few reports only on cytomegalovirus (CMV) reactivation in lupus. Diagnosis of this infection is difficult and can be associated with of a poor outcome. We report three cases of infection with CMV that occurred in patients with lupus and review the literature. CASE REPORTS: The three reported patients presented with fever, polyarthritis, myocarditis and enteritis. Lupus was longstanding and the patients were receiving corticosteroids or cyclophosphamide. There was no major CD4 lymphopenia. The diagnosis was obtained with the presence of antigenemia pp65. The outcome was favorable with antiviral therapy in two patients, while the remaining patient died. In the English literature, pulmonary and intestinal involvement seem frequent, and associated with poor prognosis. CONCLUSION: In systemic lupus CMV infection is often serious and difficult to diagnose. Risk factors, treatment and prophylaxis remain to be evaluated in this population. The incidence of this infection could increase among patients receiving a biotherapy.
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Infecciones por Citomegalovirus/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Corticoesteroides/uso terapéutico , Adulto , Antivirales/uso terapéutico , Ciclofosfamida/uso terapéutico , Infecciones por Citomegalovirus/complicaciones , Infecciones por Citomegalovirus/tratamiento farmacológico , Infecciones por Citomegalovirus/epidemiología , Femenino , Humanos , Inmunosupresores/uso terapéutico , Incidencia , Lupus Eritematoso Sistémico/tratamiento farmacológico , Persona de Mediana Edad , PronósticoRESUMEN
PURPOSE: Therapeutic education (TE) intends to help patients with systemic lupus erythematosus to better understand their disease and to improve their quality of life. The objective of this study was to assess illness perceptions of the person to provide a motivational environment for TE. METHODS: Systemic lupus erythematosus patients followed in the department of internal medicine in Lille university hospital responded to a questionnaire assessing five dimensions of the person as proposed by Giordan: the cognitive (knowledge), perceptual (fatigue and pain), affective (anxious and depressive symptoms), infra-cognitive (intimate reasoning) and metacognitive (worldview) dimensions. The quality of life was also evaluated. RESULTS: One hundred and twenty-four patients (114 women (92%); mean age 44.3±14.3 years) responded to the questionnaire. Regarding the cognitive dimension: quantity of information at the time of diagnosis was considered insufficient for 57 patients (46%). The median adherence evaluated by a scale had a median 97mm [88-100]. Regarding the perceptual dimension: pain was assessed at 59mm [44-78] and fatigue at 66mm [50-79] at visual scales. Regarding the affective dimension: prevalence of anxiety symptoms was 67% (83/124) and 28% (35/124) for depressive symptoms. Regarding the infra-cognitive dimension 78 patients (63%) had an external control place. Concerning the metacognitive dimension, systemic lupus erythematosus had repercussions on professional and family life. The quality of life was impacted. CONCLUSION: Multiple dimensions of systemic lupus erythematosus patient have to be considered for an optimal motivational environment for the practice of TE.
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Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/psicología , Adulto , Ansiedad/etiología , Estudios Transversales , Depresión/etiología , Función Ejecutiva , Fatiga/etiología , Femenino , Humanos , Masculino , Dolor/etiología , Educación del Paciente como Asunto , Calidad de Vida , Encuestas y Cuestionarios , Escala Visual AnalógicaRESUMEN
BACKGROUND: Stress might be a triggering factor causing pemphigus. We studied 11 consecutive cases of pemphigus over 5 years. OBJECTIVE: Studying and looking for a link between severe life events and the history of the disease. METHODS: An epidemiological retrospective and prospective study was carried out, including an interview and a collection of the clinical history; then the life events were integrated into the clinical history with the patient blind. Two scales were used: Paykel's inventory (assessing the negative impact of life events) and the Mini International Neuropsychiatric Interview DSM-IV (MINI). RESULTS: 10 patients out of 11 were included. With the MINI, 2 patients presented anxiety. Paykel's inventory showed type 3 life events for numerous patients, life event type 4 for 7 patients and type 5 for 3 patients, happening from 1 to 6 months before the first signs or worsening of pemphigus. We found stressful life events before the start or worsening of pemphigus for all patients with no other risk factors. CONCLUSION: Stressful life events can worsen or trigger off a pemphigus. Psychological care, associated with the immunosuppressive treatment, should entail a better management of these patients.
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Pénfigo/etiología , Estrés Psicológico/complicaciones , Adolescente , Adulto , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Pénfigo/epidemiología , Pénfigo/psicología , Pronóstico , Estudios Prospectivos , Estudios Retrospectivos , Factores de Riesgo , Estrés Psicológico/epidemiología , Estrés Psicológico/psicología , Encuestas y CuestionariosRESUMEN
Adult-onset Still's disease is a systemic disorder without specific histological feature. Diagnosis requires to rule out any other disorder including neoplasia. Nevertheless, patients with paraneoplastic adult-onset Still's disease have been reported. We report a patient with an adult-onset Still's disease who presented with a liver involvement at onset. Two years later, a liver angiosarcoma was diagnosed. This report underlines the difficulty of the diagnosis of the adult-onset Still's disease even in the presence of Yamaguchi et al.'s [J Rheumatol 19 (1992) 424-30] and Fautrel et al.'s [Medicine 81 (2002) 194-200] classification criteria and may suggest a link between the initial clinical picture and the discovery nearly two years later, of a liver angiosarcoma.
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Hemangiosarcoma/patología , Neoplasias Hepáticas/patología , Síndromes Paraneoplásicos/patología , Enfermedad de Still del Adulto/patología , Adulto , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Síndromes Paraneoplásicos/clasificación , Enfermedad de Still del Adulto/clasificaciónRESUMEN
BACKGROUND: Intravascular lymphoma is a rare form of non-Hodgkin's lymphoma characterized by proliferation of lymphoid cells within the lumen of small veins, small arteries and capillaries. CASE REPORT: A 79-year-old man presented with repeated superficial venous thrombosis of the lower limbs associated with diffuse telangiectasia of the trunk, upper arms and thighs but with normal epidermis. Screening for thrombophilia and neoplasm were negative. The patient subsequently developed abdominal pain, lower-limb oedema, deterioration in performance status and rapidly increasing telangiectasia with the appearance of generalized oedematous cutaneous induration. Increased LDH and anaemia were observed without other blood count anomalies. Intravascular lymphoma was diagnosed on a skin biopsy with telangiectasia and oedema. After eight courses of treatment with rituximab-CHOP the outcome was good. DISCUSSION: A few telangiectasias associated with nodules or infiltrated plaques are often a clinical manifestation of intravascular lymphoma. The dermatological presentation described here is interesting for two reasons: on the one hand, telangiectasias were initially isolated in normal epidermis without any infiltration for more than one year and, on the other hand, the skin infiltration seen subsequently was very extensive and marked. Moreover, although microthrombi are frequent in small and medium-sized blood vessels, thrombosis of large vessels is rarely described in intravascular lymphoma.
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Linfoma no Hodgkin/diagnóstico , Enfermedades de la Piel/etiología , Telangiectasia/diagnóstico , Trombosis de la Vena/etiología , Anciano , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales de Origen Murino , Antineoplásicos/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida/administración & dosificación , Doxorrubicina/administración & dosificación , Humanos , Linfoma no Hodgkin/tratamiento farmacológico , Linfoma no Hodgkin/patología , Masculino , Prednisona/administración & dosificación , Rituximab , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/patología , Telangiectasia/etiología , Resultado del Tratamiento , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/patología , Vincristina/administración & dosificaciónRESUMEN
INTRODUCTION: An alveolar haemorrhage occurs in 12 to 29% during microscopic polyangeitis and can reveal this disease. EXEGESIS: We report the case of a fifteen years old female patient with a microscopic polyangeitis which was diagnosed during the investigation of a chronic anemia with chronic asymptomatic alveolar haemorrhage and extracapillary glomerulonephritis with antineutrophil cytoplasmic antibodies positive (anti-myeloperoxydase antibodies). The good tolerance of alveolar haemorrhage is usual for children, particularly in idiopathic pulmonary haemorrhage but is exeptionnal for adults. According to some studies, thirty percent of idiopathic pulmonary haemorrhage goes to auto-immune disease. CONCLUSION: This observation shows that complementary pulmonary investigations are necessary in chronic anemia when gynaecologic and digestive investigations are negative and that auto-immune investigations are judicious in the survey of idiopathic pulmonary haemorrhage.
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Anemia Ferropénica/diagnóstico , Hemorragia/diagnóstico , Enfermedades Pulmonares/diagnóstico , Alveolos Pulmonares/irrigación sanguínea , Vasculitis/diagnóstico , Vasculitis/etiología , Adolescente , Biopsia , Diagnóstico Diferencial , Femenino , Humanos , Riñón/patologíaRESUMEN
INTRODUCTION: Almost 50% of patients with Wegener's granulomatosis (WG) develop ocular features, leading to visual loss in 8%. However, central retinal artery occlusion (CRAO) has exceptionally been reported. EXEGESIS: We report 2 patients with CRAO and WG according to ACR classification criteria. A literature search indicates that CRAO with WG has only been reported in 15 patients. We analyse the 17 cases to discuss systemic manifestations, ocular prognosis and treatment. CONCLUSION: CRAO is a rare manifestation of WG. Nevertheless, CRAO seems to be associated with vasculitic type of WG. So, a prompt diagnosis could lead to early aggressive regimen to improve visual and general prognosis.
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Granulomatosis con Poliangitis/complicaciones , Oclusión de la Arteria Retiniana/etiología , Anciano , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
INTRODUCTION: The cause of protein-losing enteropathy is sometimes difficult to establish. It can be rarely due to a constrictive pericarditis. EXEGESIS: We report a patient presenting a protein-losing enteropathy revealing a constrictive pericarditis. CONCLUSION: Chronic pericarditis should be evoked in case of unexplained protein-losing enteropathy. Echocardiography can sometimes be normal. Therefore, chest computed tomography scan or cardiac MRI followed by confirmation right heart catheterization should be performed in case of persistent unexplained protein-losing enteropathy.
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Pericarditis Constrictiva/complicaciones , Pericarditis Constrictiva/diagnóstico , Enteropatías Perdedoras de Proteínas/etiología , Adulto , Humanos , MasculinoRESUMEN
PURPOSE: According to current knowledge, endothelin (ET)-1 plays an important role in the pathogenesis of systemic sclerosis (SSc). We assessed ET plasma levels in SSc patients according to the clinical presentation and the presence of complications such as pulmonary arterial hypertension (PAH). METHODS: Sixty-three consecutive patients with SSc were included. The control group included 17 healthy patients. ET plasma level was determined for all patients. Pulmonary function test and pulmonary high resolution computed tomography were performed in 44 patients and echocardiography in 51 patients, to screen for PAH, always confirmed by a right heart catheterization. RESULTS: ET plasmatic levels were higher in SSc patients than in healthy group subjects but the difference was not significant (3.72+/-1.13 vs 3.40+/-0.71 pmol/l, p=0.27). ET plasmatic levels were significantly higher in patients with PAH than in patients without PAH (4.28+/-0.65 vs 3.62+/-1.07 pmol/l, p=0.04) and in patients with anticentromere antibodies (3.96+/-1.11 vs 3.19+/-1.12 pmol/l, p=0.03). There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure (r=0.34, p=0.013). The best cut-off value for ET plasmatic level to discriminate patients affected by PAH was determined by ROC curve method: 4.1 pmol/l (sensibility 85.7%, specificity 66%). CONCLUSION: ET plasmatic levels were higher in SSc patients affected by PAH and patients with anticentromere antibodies. There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure. Assessment of ET plasmatic levels for detection and monitoring of pulmonary hypertension during SSc is warranted in larger prospective studies.
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Endotelina-1/sangre , Esclerodermia Sistémica/sangre , Anciano , Biomarcadores/sangre , Cateterismo Cardíaco , Ecocardiografía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Curva ROC , Radiografía , Valores de Referencia , Esclerodermia Localizada/sangre , Esclerodermia Sistémica/diagnóstico por imagenRESUMEN
Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. A heteroquestionnaire was offered to scleroderma patients the Lille University Hospital. Data were cross-checked with the clinical database of the National Observatory of scleroderma patients. We used 104 questionnaires of which 84% are women. A total of 62.5%, (95% CI [52.5 to 71.8]) patients had experienced DP related to SSc, 55% reported symptoms worsened at work, 41% lower efficiency and a 19% job loss. Symptoms responsible for their DP were mainly asthenia, Raynaud syndrome, arthralgias and finger ulcerations. Only 40% of patients in difficulty were initiating steps with the county home for disabled people and 45% in informing their doctor at work (MT). The MT information absence was related to a misunderstanding of possible aid in 45% of cases, 57% of patients had not found a solution, 41% of them have lost their jobs and 31% of patients in the cohort experienced a drop in income. The impact of SSc the work is important. The aid job retention devices are under-used due to lack of knowledge of steps to take and the role of MT. Taking into account the symptoms responsible for PD must be improved to participate in this retention.
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Estrés Laboral/epidemiología , Ocupaciones/estadística & datos numéricos , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/epidemiología , Adaptación Fisiológica , Adulto , Anciano , Astenia/epidemiología , Estudios Transversales , Personas con Discapacidad/estadística & datos numéricos , Femenino , Francia/epidemiología , Humanos , Satisfacción en el Trabajo , Masculino , Persona de Mediana Edad , Estrés Laboral/psicología , Ocupaciones/normas , Calidad de Vida , Encuestas y CuestionariosRESUMEN
INTRODUCTION: Hyperhomocysteinemia is a biological marker that could be identified in the venous thrombotic events and rarely during acute arterial thrombotic events. The consequences can be serious. Effective diagnostic strategy is needed to optimize the management. CASE REPORT: Following bariatric surgery, a 40-year-old patient was admitted with an acute encephalopathy associated with peripheral lower limb arterial ischemia. The diagnostic work-up identified a major hyperhomocysteinemia whose causes were several. Surgical treatment and anticoagulation was associated with vitamins and trace elements supplementation. Correcting deficiencies allowed delirium and hyperhomocysteinemia improvement. Once treatment established, the patient did not present a recurrent thrombotic episode. CONCLUSION: Major hyperhomocysteinemia seems to be associated with an increased risk of acute arterial thrombosis. This marker might be considered in nutritional deficiency situations with appropriate support on the vascular, metabolic and nutrition level.
Asunto(s)
Anticoagulantes/uso terapéutico , Hiperhomocisteinemia/complicaciones , Trombosis/etiología , Adulto , Arterias/patología , Suplementos Dietéticos , Femenino , HumanosRESUMEN
BACKGROUND: Vascular thromboembolism (VTE) complicating cytomegalovirus (CMV) primary infection is increasingly reported in immunocompetent adults. No guideline is, however, currently available for the management of these infections and particularly for the antiviral therapy indication. METHODS: We performed a literature review of VTE complicating CMV primary infection in immunocompetent adults using PubMed. RESULTS: Sixty-nine case patients of VTE complicating CMV primary infection were reported. The main sites of venous thrombosis were the splanchnic veins (30 patients) or those of the lower limbs (18 patients). One-third of patients presented with pulmonary embolism (25 patients). Forty-nine patients (76%) had at least one VTE risk factor, inherited or acquired thrombophilia for 37 patients (58%), and another risk factor for 27 patients (42%). Only 11 patients received an antiviral therapy. A positive outcome was observed in all patients. CONCLUSION: We suggest that antiviral therapy should be considered for patients presenting with severe VTE, VTE with a negative outcome despite anticoagulation, severe organ involvement, or for patients managed in the intensive care unit.