RESUMEN
BACKGROUND: Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a 25 target organ in SLE. AIM: Our objective was to describe this bone marrow involvement. METHODS: This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome. Bone marrow aspirations and/or biopsies were transferred for centralized review. RESULTS: Thirty patients from 19 centers were included. Central hematologic manifestations comprised bone marrow fibrosis (n = 17; 57%), pure red cell aplasia (n = 8; 27%), myelodysplastic syndrome (n = 3; 10%), aplastic anemia and agranulocytosis (n = 1; 3% each). Bone marrow involvement was diagnosed concomitantly with SLE in 12 patients. Bone marrow biopsies showed fibrosis in 19 cases, including one case of pure red cell aplasia and one case of agranulocytosis and variable global marrow cellularity. Treatments included corticosteroids (90%), hydroxychloroquine (87%), rituximab (33%), intravenous immunoglobulins (30%), mycophenolate mofetil (20%) and ciclosporine (20%). After a median follow-up of 27 months (range: 1-142), 24 patients manifested complete improvement. No patient died. CONCLUSIONS: This registry comprises the largest series of SLE patients with bone marrow involvement. It demonstrates the strong link between SLE and bone marrow fibrosis. Patients with atypical or refractory cytopenia associated with SLE should undergo bone marrow examination to enable appropriate, and often effective, treatment. Long-term prognosis is good.
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Médula Ósea/patología , Lupus Eritematoso Sistémico/complicaciones , Pancitopenia/complicaciones , Adolescente , Adulto , Anciano , Niño , Femenino , Fibrosis , Francia , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/patología , Masculino , Persona de Mediana Edad , Pancitopenia/patología , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
Essentials Pregnancy is a risk factor for thrombosis. Management of thrombosis risk in pregnancy remains a challenge. Prophylaxis needs to be personalized. Our score may be a helpful tool for the management of pregnancies at high risk of thrombosis. SUMMARY: Background Patients with thrombophilia and/or a history of venous thromboembolism (VTE) are at risk of thrombosis during pregnancy. A risk score for pregnancies with an increased risk of VTE was previously described by our group (Lyon VTE score). Objectives The aim of this prospective study was to assess the efficacy and safety of our score-based prophylaxis strategy in 542 pregnancies managed between 2005 and 2015 in Lyon University Hospitals. Patients/Methods Of 445 patients included in the study, 36 had several pregnancies during the study period. Among these 445 patients, 279 had a personal history of VTE (62.7%), 299 patients (67.2%) had a thrombophilia marker, and 131 (29.4%) thrombophilic women had a personal history of VTE. During pregnancy, patients were assigned to one of three prophylaxis strategies according to the risk scoring system. Results In the antepartum period, low molecular weight heparin (LMWH) prophylaxis was prescribed to 64.5% of patients at high risk of VTE. Among them, 34.4% were treated in the third trimester only, and 30.1% were treated throughout pregnancy. During the postpartum period, all patients received LMWH for at least 6 weeks. Two antepartum-related VTEs (0.37%; one with a score of < 3 and the other with a score of > 6) and four postpartum-related VTEs (0.73%; three with scores of 3-5 and one with a score of > 6) occurred. No case of pulmonary embolism was observed during the study period. The rate of bleeding was 0.37%. No serious bleeding requiring transfusions or surgery occurred during the study period. Conclusion The use of a risk score may provide a rational decision process to implement safe and effective antepartum thromboprophylaxis in pregnant women at high risk of VTE.
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Anticoagulantes/administración & dosificación , Coagulación Sanguínea/efectos de los fármacos , Técnicas de Apoyo para la Decisión , Heparina de Bajo-Peso-Molecular/administración & dosificación , Complicaciones Hematológicas del Embarazo/prevención & control , Tromboembolia Venosa/prevención & control , Adulto , Anticoagulantes/efectos adversos , Toma de Decisiones Clínicas , Femenino , Francia , Hemorragia/inducido químicamente , Heparina de Bajo-Peso-Molecular/efectos adversos , Hospitales Universitarios , Humanos , Valor Predictivo de las Pruebas , Embarazo , Complicaciones Hematológicas del Embarazo/sangre , Complicaciones Hematológicas del Embarazo/diagnóstico , Complicaciones Hematológicas del Embarazo/etiología , Estudios Prospectivos , Medición de Riesgo , Factores de Riesgo , Resultado del Tratamiento , Tromboembolia Venosa/sangre , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/etiologíaRESUMEN
Systemic sclerosis (SSc) is a heterogeneous autoimmune disease associated with several antinuclear autoantibodies useful to diagnosis and prognosis. The aim of the present multicentric study was to determine the clinical relevance of antifibrillarin autoantibodies (AFA) in patients with SSc. The clinical features of 37 patients with SSc positive for AFA (AFA+) and 139 SSc patients without AFA (AFA-) were collected retrospectively from medical records to enable a comparison between AFA- and AFA+ patients. Antifibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HEp2 cells and identified by an in-house Western blot technique and/or an EliA test. Comparing AFA+ and AFA- patients, AFA+ patients were significantly younger at disease onset (36.9 versus 42.9; P = 0.02), more frequently male (P = 0.02) and of Afro-Caribbean descent (65% versus 7.7%; P < 0.001). At diagnosis, the Rodnan skin score evaluating the cutaneous manifestations was higher (13.3 versus 8.7; P = 0.01) and myositis was also more common in the AFA+ group (31.4% versus 12.2%; P < 0.01). Patients with AFA+ were not associated with diffuse cutaneous SSc or with lung involvement and no difference in survival was observed. Antifibrillarin autoantibodies are associated with patients of Afro-Caribbean origin and can identify patients with SSc who are younger at disease onset and display a higher prevalence of myositis.
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Autoanticuerpos/sangre , Proteínas Cromosómicas no Histona/inmunología , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/inmunología , Adulto , Línea Celular , Etnicidad , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Miositis/diagnóstico , Miositis/inmunología , Prevalencia , Estudios Retrospectivos , Ribonucleoproteínas Nucleolares Pequeñas/inmunología , Análisis de SupervivenciaRESUMEN
PURPOSE: To develop French recommendations about the management of vaccinations, the screening of cervical cancer and the prevention of pneumocystis pneumonia in systemic lupus erythematosus (SLE). METHODS: Thirty-seven experts qualified in internal medicine, rheumatology, dermatology, nephrology and pediatrics have selected recommendations from a list of proposition based on available data from the literature. For each recommendation, the level of evidence and the level of agreement among the experts were specified. RESULTS: Inactivated vaccines do not cause significant harm in SLE patients. Experts recommend that lupus patient should receive vaccinations accordingly to the recommendations and the schedules for the general public. Pneumococcal vaccination is recommended for all SLE patients. Influenza vaccination is recommended for immunosuppressed SLE patients. Live attenuated vaccines should be avoided in immunosuppressed patients. Yet, recent works suggest that they can be considered in mildly immunosuppressed patients. Experts have recommended a cervical cytology every year for immunosuppressed patients. No consensus was obtained for the prevention of pneumocystis pneumonia. CONCLUSION: These recommendations can be expected to improve clinical practice uniformity and, in the longer term, to optimize the management of SLE patients.
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Testimonio de Experto , Control de Infecciones/normas , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/terapia , Guías de Práctica Clínica como Asunto , Adolescente , Adulto , Francia , Humanos , Huésped Inmunocomprometido , Control de Infecciones/métodos , Infecciones/diagnóstico , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Literatura de Revisión como Asunto , Vacunación/normas , Adulto JovenRESUMEN
PURPOSE: The sarcoidosis-lymphoma syndrome is a recognised entity. However, the presence of granulomas in patients with a haematological disease should not lead too easily to a diagnosis of sarcoidosis. The presence of granulomatous lesions during the follow-up of these patients raises diagnostic and therapeutic issues. METHODS: We included 25 patients followed by the department of haematology in a French hospital (Centre Léon-Bérard). These patients presented with granulomatous lesions. Patients with a history of sarcoidosis were excluded. We report the type of haematological disease, the time of onset of the granulomatous disease compared to that of lymphoma, associated symptoms, aetiology and outcome. Patients were divided into three groups according to the time of onset of the granulomatous lesions. RESULTS: Granulomatous lesions appeared before the haematological disease in 4 cases, was concomitant in 8 cases and appeared later in 13 remaining cases. The two main subtypes of lymphoma encountered were: diffuse large cell lymphoma (36%) and Hodgkin's lymphoma (28%). Granulomatous lesions were related to the progression of the hematological disease in 11 cases, to sarcoidosis in 4 cases, to infection in 3 cases, to drug allergy in one case, to inflammatory bowel disease in one case, to granuloma annulare in one case and was isolated in 4 cases (no identified etiology). In the group where granulomas appeared after the haematological disease, mean SUV was 11 for the haematological disease versus 6.4 for granulomas. CONCLUSION: Granulomatous diseases in lymphomas can be due to various aetiologies: infection, reaction to the haematological disease, or systemic sarcoidosis. It is an important challenge for clinicians, who can miss the diagnosis of lymphoma and or conclude to a treatment failure or a relapse. Computed tomography scan (CT-scan) or (18)F-deoxyglucose-positron emission tomography scan can help establish a diagnosis but do not replace biopsy.
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Granuloma/patología , Linfoma/patología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Fluorodesoxiglucosa F18 , Francia , Granuloma/diagnóstico por imagen , Granuloma/terapia , Humanos , Linfoma/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Tomografía de Emisión de Positrones , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
OBJECTIVES: Health-related quality of life (HRQoL) has not been fully explored in antiphospholipid syndrome (APS); therefore, we compared HRQoL between APS patients and the general population and assessed the impact of thromboembolic history. METHODS: HRQoL was measured in a multicentre cohort study by the Medical Outcomes Study Short-Form 36 (MOS-SF-36) questionnaire. HRQoL scores were compared to the French general population norms. Factors significantly associated with an impaired HRQoL were identified. RESULTS: A total of 115 patients with aPL and/or systemic lupus erythematosus (SLE) were included (mean age 42.7 ± 14.1 years old, 86 women). In 53 patients APS was diagnosed. Compared to general population norms, patients with APS had an impaired HRQoL. SLE-associated APS patients had the worst HRQoL scores (physical component summary (PCS)=40.8 ± 10.6; mental component summary (MCS)=40.6 ± 16.5) in comparison with SLE or aPL patients without thromboembolic history. In APS patients, history of arterial thrombosis significantly impaired HRQoL (PCS score: 42.2 ± 9.4 vs 49.2 ± 8.5; MCS score: 33.9 ± 13.7 vs 44.6 ± 10.3). CONCLUSION: Compared to the general population, APS patients experienced a lower HRQoL. In these patients, a history of arterial thrombosis significantly impaired HRQoL. Therefore, measurements of HRQoL should be included in APS patient management to assess the burden of the disease from a patient's perspective and to provide patients with the support they need.
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Síndrome Antifosfolípido/fisiopatología , Adulto , Síndrome Antifosfolípido/psicología , Estudios de Cohortes , Femenino , Estado de Salud , Humanos , Lupus Eritematoso Sistémico/fisiopatología , Lupus Eritematoso Sistémico/psicología , Masculino , Persona de Mediana Edad , Evaluación de Resultado en la Atención de Salud , Calidad de Vida , Factores de Riesgo , Encuestas y Cuestionarios , Trombosis/fisiopatologíaRESUMEN
INTRODUCTION: Sarcoidosis is a systemic granulomatous disorder of unknown aetiology. It may rarely affect the gastrointestinal tract. CASE REPORT: We reported a 54-year-old woman with a delayed diagnosis of duodenal sarcoidosis. She presented with gastric and right upper abdominal pain associated with vomiting and marked weight loss. Abdominal computed tomographic scan showed non-compressive retroperitoneal lymph nodes and histological examination revealed non-caseating epithelioid granulomas typical of sarcoidosis. Diagnosis of duodenal sarcoidosis was obtained at the third gastroscopy. The patient's condition improved quickly with corticosteroid therapy. CONCLUSION: Gastrointestinal sarcoidosis should be looked for in patients with digestive symptoms and another sarcoid localisation. Furthermore, it is important to repeat gastroscopy to confirm diagnosis because treatment improved most patients.
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Enfermedades Duodenales/diagnóstico , Sarcoidosis/diagnóstico , Dolor Abdominal/etiología , Femenino , Gastroscopía , Humanos , Persona de Mediana Edad , Vómitos/etiología , Pérdida de PesoRESUMEN
PURPOSE: To develop French recommendations about screening and management of cardiovascular risk factors in systemic lupus erythematosus (SLE). METHODS: Thirty-nine experts qualified in internal medicine, rheumatology and nephrology have selected recommendations from a list developed based on evidence from the literature. For each recommendation, the level of evidence and the level of agreement among the experts were specified. RESULTS: Experts recommended an annual screening of cardiovascular risk factors in SLE. Statins should be prescribed for primary prevention in SLE patients based on the level of LDL-cholesterol and the number of cardiovascular risk factors, considering SLE as an additional risk factor. For secondary prevention, experts have agreed on an LDL-cholesterol target of <0.7 g/L. Hypertension should be managed according to the 2013 European guidelines, using renin-angiotensin system blockers as first line agents in case of renal involvement. Aspirin can be prescribed in patients with high cardiovascular risk or with antiphospholipid antibodies. CONCLUSION: These recommendations about the screening and management of cardiovascular risk factors in SLE can be expected to improve clinical practice uniformity and, in the longer term, to optimize the management of SLE patients.
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Enfermedades Cardiovasculares/etiología , Lupus Eritematoso Sistémico/complicaciones , Tamizaje Masivo/métodos , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/tratamiento farmacológico , Medicina Basada en la Evidencia , Testimonio de Experto , Guías como Asunto , Humanos , Factores de Riesgo , Prevención SecundariaRESUMEN
Autoimmune diseases may reveal or occur during the course of a neoplasia or its treatment. Autoimmune cytopenia, especially haemolytic anaemia, is common in lymphoproliferative disorders such as chronic lymphoid leukemia. The link between cancer and myositis is well established. Dermatomyositis is associated with an increased relative risk of cancer of 3.4 to 4.4. A combination of detection of antibodies against p155 and TEP-computed tomography may be the best approach to ascertain the presence of occult malignancy in patients with dermatomyositis. A cutaneous or a systemic vascularitis may reveal a cancer, most often a haematological malignancy such as hairy cell leukemia. Paraneoplastic polyarthritis have been described in particular with adenocardinoma of the lungs. Underlying neoplasia should be considered in male smokers patients with new onset polyarthritis and poor health status. The prevalence of autoimmune conditions in myelodysplastic syndromes is 10 to 30%. Vasculitis and relapsing polychondritis are the most commonly reported manifestations. Immune manifestations can also be related to treatment. The most common treatment complications are autoimmune haemolytic anaemia with fludarabine and thyroiditis related to interferon and cervical radiotherapy.
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Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/terapia , Neoplasias/complicaciones , Neoplasias/terapia , Antineoplásicos/efectos adversos , Enfermedades Autoinmunes/inducido químicamente , Humanos , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/inmunología , Masculino , Síndromes Mielodisplásicos/complicaciones , Síndromes Mielodisplásicos/inmunología , Miositis/complicaciones , Miositis/inmunología , Enfermedades Reumáticas/complicaciones , Enfermedades Reumáticas/inmunología , Vasculitis/complicaciones , Vasculitis/inmunologíaAsunto(s)
Factor V/genética , Heterocigoto , Mutación , Protrombina/genética , Embolia Pulmonar/genética , Tromboembolia Venosa/genética , Trombosis de la Vena/genética , Adulto , Femenino , Francia/epidemiología , Predisposición Genética a la Enfermedad , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Fenotipo , Valor Predictivo de las Pruebas , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/epidemiología , Estudios Retrospectivos , Factores de Riesgo , Tomografía Computarizada Espiral , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/epidemiología , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/epidemiologíaRESUMEN
PURPOSE: Infectious aortic aneurysms are rare conditions, being responsible of 2% of aortic aneurysms. Most published results are surgical case series concerning infected abdominal aorta. In this retrospective study, we assessed clinical features and outcome of patients presenting infectious thoracic aortic aneurysms. PATIENTS AND METHODS: Diagnosis was based upon a combination of imaging evidence for thoracic aorta aneurysm and evidence for an infective aetiology including a culture of a causative pathogen, or a favourable outcome with anti-infective therapy. Retrospective case series. RESULTS: Six men and one woman were included, with a mean age of 66 years. All the patient presented at least one cardiovascular risk factor or atherosclerosis localisation. Fever (71%) and chest pain (42%) were the most common clinical presenting manifestations. The causative pathogens were: Staphylococcus aureus (N=1), Salmonella enteritidis (N=3) and Candida albicans (N=1). The contrast-enhanced computed-tomography disclosed an aneurysm whose diameter reached more than 50 mm (N=5), that increased rapidly in size (N=5), or presented an inflammatory aspect of the aortic wall (N=4). Management was both medical and interventional: surgery (N=3) or endoluminal repair (N=4). Outcome was favourable in six patients; one patient died from aneurysm-related complications. CONCLUSION: Clinical manifestations revealing an infectious thoracic aneurysm are variable. Diagnosis should be considered in patients presenting a rapidly-growing aneurysm, especially in the presence of elevated acute phase reactants. Endoluminal repair constitutes a treatment option. The role of FDG-PET for diagnosis and follow-up remains to be defined.
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Aneurisma Infectado/microbiología , Aneurisma Infectado/cirugía , Aneurisma de la Aorta Torácica/diagnóstico , Aneurisma de la Aorta Torácica/cirugía , Anciano , Anciano de 80 o más Años , Aneurisma Infectado/diagnóstico , Aneurisma Infectado/mortalidad , Antibacterianos/uso terapéutico , Aneurisma de la Aorta Torácica/mortalidad , Prótesis Vascular , Candidiasis/complicaciones , Candidiasis/tratamiento farmacológico , Dolor en el Pecho/etiología , Femenino , Fiebre/etiología , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Infecciones por Salmonella/complicaciones , Infecciones por Salmonella/tratamiento farmacológico , Infecciones Estafilocócicas/complicaciones , Infecciones Estafilocócicas/tratamiento farmacológicoRESUMEN
The link between systemic disease and cancer is not fortuitous. An autoimmune disease can represent the starter for developing a non-Hodgkin lymphoma. This is particularly true for Sjögren's syndrome that is associated with the highest risk of lymphoma (odds ratio up to 44). Other systemic autoimmune diseases concerned are systemic lupus with an odds ratio of 4.5 and rheumatoid arthritis with an odds ratio of 2 to 3. It is now well established that high inflammatory activity, rather than immunosuppressive treatment, is the major risk determinant. The association between solid cancer and autoimmune systemic disease is uncommon and concerns in particular scleroderma and lung cancer. Concerning biotherapy-induced cancers, there is no demonstrated increased risk with anti-TNFα (except for cutaneous carcinoma and maybe melanoma) or with tocilizumab and abatacept even if studies with longer follow-up are needed at least for these two last drugs.
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Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/terapia , Neoplasias/etiología , Neoplasias/terapia , Enfermedades Autoinmunes/inmunología , Productos Biológicos/efectos adversos , Transformación Celular Neoplásica/efectos de los fármacos , Transformación Celular Neoplásica/inmunología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/inmunología , Humanos , Inmunosupresores/efectos adversos , Linfoma/complicaciones , Linfoma/inmunología , Neoplasias/inducido químicamente , Neoplasias/inmunologíaRESUMEN
AIM: We hypothezised that patients (cases) who are hospitalized for a major ischemic event--myocardial infarction, stroke, decompensation of peripheral arterial disease--acquire better knowledge than a control population--atheromatous patients without a major ischemic event, patients consulting for a vein disease or a diabetes evaluation, and accompanists--about cardiovascular risk factors (smoking, hypertension, diabetes, dyslipidemia, obesity) and have a better understanding of the usefulness of making changes in their lifestyle (quit smoking, regular exercise, Mediterranean diet, low salt diet, weight control, diabetes care). METHODS: A questionnaire was proposed at vascular surgery consultations and vascular and cardiac functional explorations, at the M Pavillon of the Édouard-Herriot hospital, Lyon, France. In five months, 395 questionnaires (135 cases and 260 controls) were analyzed. RESULTS: The global knowledge score was statistically higher for cases than for controls (cases 3.23±1.81; controls 2.77±2.03; P=0.037). Cases did not abide by monitoring and dietary rules better, except as regards the management of diabetes. Regular physical activity was statistically more prevalent among controls than among cases. Cases mainly received their information from their doctors (general practitioner for 59% of controls and 78% of cases, cardiologist for 25% of controls and 57% of cases) while controls got their information more through magazines or advertising. CONCLUSION: Our results show that after a major ischemic event, cases' knowledge of risk factors is better than the rest of the population without improved rules lifestyle changes. This suggests the usefulness of evaluating a therapeutic education program for atheromatous disease.
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Enfermedades Cardiovasculares/etiología , Conocimientos, Actitudes y Práctica en Salud , Infarto del Miocardio , Enfermedad Arterial Periférica , Accidente Cerebrovascular , Enfermedades Cardiovasculares/prevención & control , Complicaciones de la Diabetes , Dieta/efectos adversos , Dieta Mediterránea , Dieta Hiposódica , Dislipidemias/complicaciones , Ejercicio Físico , Humanos , Hipertensión/complicaciones , Estilo de Vida , Obesidad/complicaciones , Educación del Paciente como Asunto , Estudios Prospectivos , Factores de Riesgo , Fumar/efectos adversos , Encuestas y CuestionariosRESUMEN
A 4 year old girl is referred to our institution for resection of a nephroblastoma with an extension of the tumor into the lumen of the inferior vena cava. To perform a correct resection of the tumor, the operation was conducted under cardiopulmonary bypass. At the end of the procedure, a bilateral mydriasis was noticed. A CT-scan concluded to a massive venous air emboli. As the procedure was unventful, and no other cause of air emboli was found, the etiology of this emboli is problably retrograde because of the large opening of the inferior vena cava that was required to remove the tumor. To avoid similar case the use of transcranial doppler monitoring may be of interest.
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Embolia Aérea/etiología , Complicaciones Intraoperatorias/etiología , Neoplasias Renales/cirugía , Vena Cava Inferior/cirugía , Tumor de Wilms/cirugía , Preescolar , Femenino , Humanos , Neoplasias Renales/patología , Células Neoplásicas Circulantes , Tumor de Wilms/patologíaAsunto(s)
Trombosis de la Vena/diagnóstico , Trombosis de la Vena/terapia , Abdomen/irrigación sanguínea , Venas Cerebrales/patología , Humanos , Trombosis Intracraneal/diagnóstico , Trombosis Intracraneal/patología , Trombosis Intracraneal/terapia , Extremidad Inferior/irrigación sanguínea , Pelvis/irrigación sanguínea , Oclusión de la Vena Retiniana/diagnóstico , Oclusión de la Vena Retiniana/terapia , Venas/patología , Trombosis de la Vena/complicacionesRESUMEN
INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder. Drug-induced TTP is uncommon and we report a TTP associated with the use of clopidogrel. CASE REPORT: We report a 50-year-old man who presented with acute myocardial infarction and received clopidogrel therapy. He developed acute TTP ten days after clopidogrel onset. Imputability of the drug was demonstrated during a reintroduction test. Deficiency of ADAMTS 13 was confirmed and autoantibodies against ADAMTS 13 were detected. Complete remission was obtained after 24 plasma exchange sessions and adjunction of corticosteroids. CONCLUSION: Drug-induced TTP are probably immunologic, as was demonstrated in our patient. Clinicians should be aware of this possible uncommon adverse effect of clopidogrel because prompt therapy is imperative for life saving.
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Púrpura Trombocitopénica Trombótica/inducido químicamente , Púrpura Trombocitopénica Trombótica/diagnóstico , Ticlopidina/análogos & derivados , Clopidogrel , Humanos , Masculino , Persona de Mediana Edad , Infarto del Miocardio/tratamiento farmacológico , Inhibidores de Agregación Plaquetaria/efectos adversos , Inhibidores de Agregación Plaquetaria/uso terapéutico , Ticlopidina/efectos adversos , Ticlopidina/uso terapéuticoRESUMEN
PURPOSE: Thrombotic events may occur in celiac disease. In this study, we analyzed clinical features and risk factors for thrombosis in seven patients who had celiac disease and thrombosis. METHODS: We retrospectively studied 87 patients with adult celiac disease and identified seven cases of thrombosis. We searched if risk factors for thrombosis were identified and tested retrospectively antiphospholipid antibodies on the serum. RESULTS: In our study, the global prevalence of thrombosis was 8 %, and 5.7 % for spontaneous thrombosis, with venous thrombosis (n=5) or arterial thrombosis (n=1) or both (n=2). The seven patients consisted in six women and one man with a mean age of 44.8 years at time of thrombosis. Thrombotic events occurred before the diagnosis of celiac disease in four cases. In three cases, venous thrombosis was in unusual sites: portal (n=2), splenic vein thrombosis (n=1). In six cases, we identified risk factors for thrombosis, which could be linked to celiac disease: hyperhomocysteinemia (n=1), protein C and S deficiency due to vitamin K deficiency (n=3) and antiphospholipid antibodies (n=2). CONCLUSION: Such risk factors for thrombosis should be identified in patients in adult celiac disease in order to correct them and add a thromboembolic prophylaxis.
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Enfermedad Celíaca/complicaciones , Trombosis/complicaciones , Adulto , Anticuerpos Antifosfolípidos/sangre , Femenino , Humanos , Hiperhomocisteinemia/complicaciones , Masculino , Persona de Mediana Edad , Deficiencia de Proteína C/complicaciones , Deficiencia de Proteína S/complicaciones , Estudios Retrospectivos , Factores de Riesgo , Deficiencia de Vitamina K/complicacionesRESUMEN
Esophageal involvement occurs in about 80% of patients with systemic sclerosis, with a marked diminution of peristaltic pressures in the distal two-thirds of the esophagus. Our aims were to more fully characterize esophageal motility disorders in systemic sclerosis using high-resolution manometry (HRM) and to determine predictive factors of esophageal involvement. Fifty-one patients (46 females) with systemic sclerosis were included in this retrospective study. Esophageal motility was characterized with HRM. The demographic data, esophageal symptoms, presence of other organ involvement, and autoantibody profile (anti-Scl70 antibodies [Scl70], anticentromere antibodies [ACA]) were recorded for all patients. Esophageal body dysmotility was present in 33 patients (67.3%) and was associated with hypotensive esophagogastric junction in 27 patients (55.1%). The velocity of proximal contractions was higher in patients with esophageal body dysmotility compared to patients with normal peristalsis (median 10.8 cm/s vs. 5.5, P = 0.04). The amplitude of middle esophageal contraction but not of distal esophageal contraction was reduced in patients with hypoperistalsis. Diffuse esophageal skin involvement, presence of Scl70 and absence of ACA were associated with esophageal involvement. Esophageal symptoms encountered in 87.5% of patients were not predictive of esophageal dysmotility. This HRM series confirms the high prevalence of esophageal body dysmotility in systemic sclerosis. Diffuse skin involvement, positive Scl70 and negative ACA, but not esophageal symptoms, may predict esophageal body dysmotility.
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Trastornos de la Motilidad Esofágica/fisiopatología , Manometría/métodos , Esclerodermia Sistémica/complicaciones , Adulto , Anciano , Anciano de 80 o más Años , Anticuerpos Antinucleares/sangre , Autoanticuerpos/sangre , Trastornos de la Motilidad Esofágica/epidemiología , Trastornos de la Motilidad Esofágica/etiología , Unión Esofagogástrica/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Peristaltismo , Prevalencia , Estudios Retrospectivos , Esclerodermia Sistémica/sangre , Adulto JovenRESUMEN
We report a 39-year-old woman with systemic lupus who presented with recurrent aseptic meningitis secondary to treatment with nonsteroidal anti-inflammatory drugs (NSAIDs). She presented two episodes following ibuprofen administration that were characterized by aseptic meningitis with high protein level in cerebrospinal fluid, and increased serum acute phase reactants. No evidence of an infection or vasculitis was documented. Clinical manifestation resolved rapidly with ibuprofen discontinuation, and corticosteroids therapy was unnecessary. Aseptic meningitis related to NSAIDs reported in lupus patients should be considered because of their specific modality of care and their favourable outcome.