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1.
Rev Med Interne ; 45(3): 126-131, 2024 Mar.
Artigo em Francês | MEDLINE | ID: mdl-38355359

RESUMO

BACKGROUND: Anti-centromere antibodies, anti-topoisomerase-1 antibodies (ATA), and anti-RNA-polymerase III antibodies are three Systemic Sclerosis (SSc)-specific autoantibodies. Their detection is helpful in determining the prognosis. We aimed to evaluate whether ATA levels were associated with disease severity at diagnosis or disease progression during follow-up in ATA positive patients. METHODS: We conducted a single-centre French retrospective observational study, between 2014 and 2021. ATA positive patients fulfilling the ACR/EULAR 2013 classification criteria for SSc with a minimal follow-up of 1 year and 2 ATA dosages were included. SSc patients with high IgG ATA levels at baseline (>240IU/mL) were compared with SSc patients with low levels (≤240IU/mL), at inclusion and at 1 and 3 years. A variation of at least 30 % of ATA levels was considered significant. RESULTS: Fifty-nine SSc patients were included and analysed. There was a predominance of women and of patients with diffuse interstitial lung disease. Patients with high ATA levels exhibited a higher skin sclerosis assessed by the modified Rodnan skin score (P=0.0480). They had a lower carbon monoxide transfer coefficient (P=0.0457), a lower forced vital capacity (FVC) (P=0.0427) and more frequently had a FVC under 80 %, when compared to patients with low ATA levels (P=0.0423). Initial high ATA levels were associated with vascular progression at one year (21.95 % vs. 0 %; P=0.0495). CONCLUSION: ATA levels are associated with skin sclerosis and vascular progression in SSc. Beyond the detection of ATA, quantifying this autoantibody might be of interest in predicting disease severity and prognosis in SSc.


Assuntos
Autoanticorpos , Escleroderma Sistêmico , Humanos , Feminino , Masculino , Autoanticorpos/análise , Esclerose/complicações , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Prognóstico , Fibrose
2.
Rev Med Interne ; 44(5): 212-217, 2023 May.
Artigo em Francês | MEDLINE | ID: mdl-37029032

RESUMO

INTRODUCTION: Infections are associated with morbimortality of patients with giant cell arteritis (GCA). The aim of this work was twofold: the identification of factors predisposing to the risk of infection and the description of patients hospitalized with an infection occurring during the treatment period of CAG. METHODS: A monocentric retrospective study was conducted in GCA patients, comparing patients hospitalized for infection with patients without infection. The analysis included 21/144 (14.6%) patients with 26 infections (cases) and 42 control matched on sex, age, and diagnosis of GCA. RESULTS: Both groups were similar except for a higher frequency of seritis in cases (15% vs. 0%, p=0.03). Relapses of GCA were less common in cases (23.8% vs 50.0%, p=0.041). Hypogammaglobulinemia was present during infection. More than half of the infections (53.8%) occurred in the first year of follow-up with an average dose of 15mg/day of corticosteroids. Infections were mainly pulmonary (46.2%) and cutaneous (26.9%). CONCLUSION: Factors associated with infectious risk were identified. This preliminary monocentric work will continue with a national multicentre study.


Assuntos
Arterite de Células Gigantes , Humanos , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/epidemiologia , Estudos Retrospectivos , Glucocorticoides/uso terapêutico , Corticosteroides/uso terapêutico , Hospitalização
3.
Rev Med Interne ; 44(5): 227-252, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37147233

RESUMO

This French National Diagnostic and Care Protocol (NDPC) includes both pediatric and adult patients with non-infectious chronic uveitis (NICU) or non-infectious recurrent uveitis (NIRU). NICU is defined as uveitis that persists for at least 3 months or with frequent relapses occurring less than 3 months after cessation of treatment. NIRU is repeated episodes of uveitis separated by periods of inactivity of at least 3 months in the absence of treatment. Some of these NICU and NIRU are isolated. Others are associated with diseases that may affect various organs, such as uveitis associated with certain types of juvenile idiopathic arthritis, adult spondyloarthropathies or systemic diseases in children and adults such as Behçet's disease, granulomatoses or multiple sclerosis. The differential diagnoses of pseudo-uveitis, sometimes related to neoplasia, and uveitis of infectious origin are discussed, as well as the different forms of uveitis according to their main anatomical location (anterior, intermediate, posterior or panuveitis). We also describe the symptoms, known physiopathological mechanisms, useful complementary ophthalmological and extra-ophthalmological examinations, therapeutic management, monitoring and useful information on the risks associated with the disease or treatment. Finally, this protocol presents more general information on the care pathway, the professionals involved, patient associations, adaptations in the school or professional environment and other measures that may be implemented to manage the repercussions of these chronic diseases. Because local or systemic corticosteroids are usually necessary, these treatments and the risks associated with their prolonged use are the subject of particular attention and specific recommendations. The same information is provided for systemic immunomodulatory treatments, immunosuppressive drugs, sometimes including anti-TNFα antibodies or other biotherapies. Certain particularly important recommendations for patient management are highlighted in summary tables.


Assuntos
Síndrome de Behçet , Esclerose Múltipla , Uveíte , Adulto , Humanos , Criança , Uveíte/diagnóstico , Uveíte/epidemiologia , Uveíte/etiologia , Síndrome de Behçet/complicações , Corticosteroides/uso terapêutico , Imunossupressores/uso terapêutico , Esclerose Múltipla/complicações
4.
Eur J Clin Microbiol Infect Dis ; 31(3): 365-9, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21785920

RESUMO

Diplorickettsia massiliensis has been recently isolated from Ixodes ricinus ticks. We screened 13,872 serum samples from patients in France with suspected tick-borne diseases and found three cases that had serological evidence of D. massiliensis infection; for one of these three cases, we obtained molecular evidence of an infection as well.


Assuntos
Coxiellaceae/classificação , Coxiellaceae/patogenicidade , Infecções por Rickettsia/diagnóstico , Infecções por Rickettsia/patologia , Adulto , Idoso , Animais , Anticorpos Antibacterianos/sangue , DNA Bacteriano , Feminino , Humanos , Ixodes/microbiologia , Masculino , Camundongos , Camundongos Endogâmicos BALB C , Pessoa de Meia-Idade , Doenças Transmitidas por Carrapatos/diagnóstico , Doenças Transmitidas por Carrapatos/patologia
5.
Rev Med Interne ; 42(3): 202-209, 2021 Mar.
Artigo em Francês | MEDLINE | ID: mdl-33455838

RESUMO

Immune Thrombotic Thrombocytopenic Purpura (iTTP) is a rare but severe disease with a mortality rate of almost 100 % in the absence of adequate treatment. iTTP is caused by a severe deficiency in ADAMTS13 activity due to the production of inhibitory antibodies. Age has been shown to be a major prognostic factor. iTTP patients in the elderly (60yo and over) have more frequent organ involvement, especially heart and kidney failures compared with younger patients. They also have non-specific neurologic symptoms leading to a delayed diagnosis. Factors influencing this impaired survival among older patients remain unknown so far. Alteration of the functional capacity of involved organs could be part of the explanation as could be the consequences of vascular aging. In fact, severe ADAMTS13 deficiency is necessary but likely not sufficient for iTTP physiopathology. A second hit leading to endothelial activation is thought to play a central role in iTTP. Interestingly, the mechanisms involved in endothelial activation may share common features with those involved in vascular aging, potentially leading to endothelial dysfunction. It could thus be interesting to better investigate the causes of mid- and long-term mortality among older iTTP patients to confirm whether inflammation and endothelial activation really impact vascular aging and long-term mortality in those patients, in addition to their presumed role at iTTP acute phase. If so, further insights into the mechanisms involved could lead to new therapeutic targets.


Assuntos
Púrpura Trombocitopênica Idiopática , Púrpura Trombocitopênica Trombótica , Doenças Vasculares , Proteína ADAMTS13 , Idoso , Humanos , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/epidemiologia , Púrpura Trombocitopênica Trombótica/terapia
6.
J Fr Ophtalmol ; 44(2): 252-258, 2021 Feb.
Artigo em Francês | MEDLINE | ID: mdl-33423814

RESUMO

Immunosuppressants are prescribed for pediatric uveitis in cases of severe involvement affecting the prognosis for vision or life, in cases of recurrent or chronic uveitis to achieve corticosteroid sparing, or in cases of corticosteroid resistance. Immunosuppressants used in children include antimetabolites (methotrexate, mycophenolate mofetil, azathioprine), cyclosporine, tacrolimus, and biologics, including infliximab, adalimumab, anakinra, canakinumab, and tocilizumab. The mechanisms of action and indications of the various immunosuppressants are described in this review.


Assuntos
Imunossupressores , Uveíte , Adalimumab , Criança , Humanos , Imunossupressores/uso terapêutico , Infliximab , Ácido Micofenólico , Uveíte/tratamento farmacológico
7.
Rev Med Interne ; 41(7): 440-445, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32063422

RESUMO

Among the antibodies described in Systemic Sclerosis (SSc), anti-Th/To antibodies (anti-Th/To) are rare and have been poorly studied. Thus, little is known about the profile of anti-Th/To positive patients. From our local Biobank (Marseille, France), we retrospectively selected data for 6 patients positive for anti-Th/To with an Immunodot assay. All of them suffered from SSc, sharing clinical and biological common features such as a limited cutaneous form of SSc, a decreased lung diffusing capacity and a speckled nuclear nucleolar immunofluorescence pattern of antinuclear antibodies screening on HEp-2 cells. In order to further characterize patients positive for anti-Th/To, we performed a thorough literature review. From 402 studied patients positive for anti-Th/To, we confirmed that these antibodies are associated with the limited cutaneous form of the disease (88% of the patients), and with an SSc related-pulmonary involvement (50%). The review analysis pointed out the rarity of the anti-Th/To with an estimated mean frequency of 3.4% of all SSc patients worldwide, their usual exclusivity with respect to the specific antibodies of scleroderma, and their high specificity (around 98%) for the diagnosis of SSc.


Assuntos
Anticorpos Antinucleares/sangue , Autoanticorpos/sangue , Endorribonucleases/imunologia , Ribonucleoproteínas/imunologia , Escleroderma Sistêmico/sangue , Idoso , Diagnóstico Diferencial , Feminino , França , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/imunologia
8.
Rev Med Interne ; 30(3): 208-14, 2009 Mar.
Artigo em Francês | MEDLINE | ID: mdl-18992971

RESUMO

PURPOSE: During bone-marrow biopsy, one third of patients score their pain as moderate or severe. Combination of analgesic and hypnotic is effective at reducing pain, but prolonged medical surveillance is necessary. The objective of the study was to assess the effectiveness and ease of use of the equimolar nitrous oxide-oxygen mixture (nitrous oxide), a short acting analgesic with little sedative effect, during bone-marrow biopsy. METHODS: As part of a non-controlled prospective observational study, patients undergoing a bone-marrow biopsy received nitrous oxide as an adjuvant to local anaesthesia. Facemask was self-maintained. A questionnaire was given after completion of the procedure to assess the pain (with a numerical-rating scale [RS] ranging from 0 to 10) as the main criterion and the ease of the procedure, the tolerance, and satisfaction, as secondary criteria. The physician noted adverse reactions. RESULTS: Nineteen women and 21 men were included. The median age was 51 years. Ninety percent of patients felt slight pain, less than 5 out of 10 on the RS. Only one patient had difficulty in keeping the mask. A patient experienced nausea and four presented a fleeting euphoria noticed by the physician. Ninety-five of patients wished to use nitrous oxide again should further bone-marrow examinations be necessary. CONCLUSION: Nitrous oxide is an effective analgesic when performing bone-marrow biopsies. Ten percent of patients feel a moderate to severe pain instead of one third. Despite some mild side effects, there is a very good appreciation by patients. Since this study, the authors routinely use nitrous oxide.


Assuntos
Analgésicos não Narcóticos/administração & dosagem , Biópsia por Agulha/efeitos adversos , Exame de Medula Óssea , Medula Óssea/patologia , Óxido Nitroso/administração & dosagem , Oxigênio/administração & dosagem , Dor/etiologia , Dor/prevenção & controle , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Interpretação Estatística de Dados , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Inquéritos e Questionários
9.
Rev Med Interne ; 40(5): 278-285, 2019 May.
Artigo em Francês | MEDLINE | ID: mdl-30573331

RESUMO

PURPOSE: Giant cell arteritis (GCA) is the most common vasculitis of the elderly. In order to assess the impact of age at diagnosis, we compared the characteristics of patients of less than 75 years (<75 years), to those of the 75 years and over (≥75 years). PATIENTS AND METHODS: We conducted a retrospective study on 164 patients with GCA diagnosed from 2005 to 2017. All patients had at least 3/5 of the ACR criteria and had a CT-scan at diagnosis. The mean age was of 73±9.6 years. The age was<75 years for 84 patients (59 women) and≥75 years for 80 patients (53 women). RESULTS: Patients≥75 years had more cardiovascular underlying diseases (P=0.026), a higher rate of hypertension (P=0.005) and more ophthalmic complications (P=0.02). They had less large vessel involvement (P<0.001), showed lower biological inflammatory reaction and had a more frequently positive temporal artery histology (P=0.04). The oral initial dose of corticosteroids did not differ between the groups. Corticosteroids pulse therapy was more frequent in patients≥75 years (P=0.01). The frequency of anti-platelet agents use was similar in the two groups. Relapse rate, corticodependance and the rate of corticosteroids weaning were similar in both groups. CONCLUSION: Patients≥75 years at diagnosis of GCA were at lower risk of aortitis but were more likely to suffer from ophthalmic complications and to receive corticosteroid pulse therapy.


Assuntos
Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/terapia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
10.
Rev Med Interne ; 29(10): 794-800, 2008 Oct.
Artigo em Francês | MEDLINE | ID: mdl-18023939

RESUMO

PURPOSE: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder and early treatment is vital. Here, we review the recent advances in the understanding of the pathophysiology of TTP and its treatment. CURRENT KNOWLEDGE AND KEY POINTS: Recent advances have shown that TTP is caused by deficiency of the (ADAMTS-13) metalloprotease that cleaves von Willebrand factor multimers. Acquired TTP is associated to inhibitory antibodies directed against ADAMTS-13. This has led to assess new therapeutic approaches in refractory and relapsing forms of TTP and the use of rituximab has shown very encouraging results. FUTURE PROSPECTS AND PROJECTS: A better characterization of TTP amongst the other thrombotic microangiopathies has allowed the use of new therapeutic approaches with the use of rituximab. The encouraging results reported with rituximab in some forms of TTP challenge the classic treatment based on plasma exchanges.


Assuntos
Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Corticosteroides/uso terapêutico , Diagnóstico Diferencial , Humanos , Troca Plasmática , Inibidores da Agregação Plaquetária/uso terapêutico , Púrpura Trombocitopênica Trombótica/epidemiologia , Púrpura Trombocitopênica Trombótica/fisiopatologia
11.
Rev Med Interne ; 39(9): 721-727, 2018 Sep.
Artigo em Francês | MEDLINE | ID: mdl-29933971

RESUMO

Retinal vasculitis (RV) is an inflammation of retinal blood vessels that can be associated with uveitis or be isolated, and can induce vascular occlusion and retinal ischemia. Visual acuity can be severely affected in case of macular involvement or neovessel formation. The diagnosis relies on fundoscopy and fluorescein angiography. Systemic diseases may be associated with RV, the most frequently encountered are Behçet's disease, sarcoidosis or multiple sclerosis, all predominantly associated with venous involvement, whereas systemic lupus erythematosus and necrotizing vasculitis are less frequently observed and predominantly associated with arterial or mixed vasculitis. Treatments are usually aggressive in order to preserve a good visual acuity and to reduce retinal inflammation and chronic ischemia. Steroids, immunosuppressive drugs, retinal laser photocoagulation, intravitreal anti-VEGF injections are usual treatments and more recently, anti-TNFalpha monoclonal therapeutic antibodies have been shown to be very successful.


Assuntos
Doenças Autoimunes , Inflamação , Vasculite Retiniana , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/terapia , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/terapia , Humanos , Inflamação/complicações , Inflamação/diagnóstico , Inflamação/terapia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/terapia , Vasculite Retiniana/diagnóstico , Vasculite Retiniana/etiologia , Vasculite Retiniana/terapia , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/terapia
12.
Rev Med Interne ; 39(9): 676-686, 2018 Sep.
Artigo em Francês | MEDLINE | ID: mdl-29122311

RESUMO

INTRODUCTION: Diagnostic work-up of uveitis involves many uncertainties. Search for an etiology should take into account the epidemiology of uveitis and focus on the most severe diseases or those, which can be treated. This work was undertaken to establish recommendations for the diagnosis work-up of uveitis. METHODS: Recommendations were developed by a multidisciplinary panel of 15 experts, including internists, ophthalmologists and a rheumatologist and are based on a review of the literature with regard to effectiveness of investigations and the results of the ULISSE study, which is the first prospective study assessing the efficiency of a standardized strategy for the etiological diagnosis of uveitis. Children, immunocompromised patients, severe retinal vasculitis and specific ophthalmological entities are excluded from these recommendations. RESULTS: Investigations should be first guided by the history and physical examination. Serological screening for syphilis is the only test appropriate in all forms of uveitis. If no diagnosis is made after this stage, we propose investigations guided by the anatomic characteristics of uveitis. It includes HLA B27 testing (in unilateral acute anterior non-granulomatous uveitis), serum angiotensin converting enzyme, interferon-gamma release assay and chest CT (chronic uveitis), cerebral MRI and anterior chamber tap with IL10 analysis (intermediate or posterior uveitis in patients over 40 years). Investigations ordered in the absence of orientation are almost always unhelpful. CONCLUSIONS: We propose a strategy for the etiologic diagnosis of uveitis. The recommendations should be updated regularly. The efficiency of more invasive investigations has yet to be evaluated.


Assuntos
Técnicas de Diagnóstico Oftalmológico/normas , Guias de Prática Clínica como Assunto , Uveíte/diagnóstico , Adulto , Criança , Prova Pericial , Humanos , Programas de Rastreamento
13.
Rev Med Interne ; 39(9): 711-720, 2018 Sep.
Artigo em Francês | MEDLINE | ID: mdl-29496270

RESUMO

Scleritis is an inflammatory disease of the sclera; outer tunic of the eye on which the oculomotor muscles are inserted. It can be associated with a systemic disease up to one time out of 3. These associated diseases are mainly rheumatoid arthritis, vasculitis, including granulomatosis with polyangiitis in the first line and spondyloarthropathies. Before mentioning such an etiology, it is necessary to eliminate an infectious cause, mainly herpetic, which is regularly underestimated. The classification of scleritis is clinical. We distinguish between anterior scleritis and posterior scleritis. Anterior scleritis is diffuse or nodular, usually of good prognosis. Anterior necrotizing scleritis with inflammation is often associated with an autoimmune disease, necrotizing scleritis without inflammation usually reflects advanced rheumatoid arthritis. The treatment of these conditions requires close collaboration between internists and ophthalmologists to decide on the use of corticosteroid therapy with or without immunosuppressors or biotherapies.


Assuntos
Doenças Autoimunes , Conhecimentos, Atitudes e Prática em Saúde , Inflamação , Medicina Interna/métodos , Médicos , Esclerite , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/terapia , Técnicas de Diagnóstico Oftalmológico , Humanos , Inflamação/complicações , Inflamação/diagnóstico , Inflamação/terapia , Esclerite/diagnóstico , Esclerite/etiologia , Vasculite/complicações , Vasculite/diagnóstico , Vasculite/terapia
14.
Rev Med Interne ; 39(9): 687-698, 2018 Sep.
Artigo em Francês | MEDLINE | ID: mdl-29610003

RESUMO

Conventional immunosuppressive drugs, anti-TNF alpha and other biotherapies used in clinical practice are capable of controlling non-infectious anterior uveitis, posterior uveitis and panuveitis. The present work has been led by a multidisciplinary panel of experts, internists, rheumatologists and ophthalmologists and is based on a review of the literature. In case of corticodependency or sight-threatening disease, conventional immunosuppressive drugs (methotrexate, azathioprine and mycophenolate mofetil) and/or anti-TNF alpha (adalimumab, infliximab) are used to achieve and maintain remission. Interferon is an efficient immunomodulatory treatment, as a second-line therapy, for some therapeutic indications (refractory macular edema, Behçet's vascularitis). Other biologics, especially tocilizumab, are showing promising results. Local treatments (corticosteroids, sirolimus etc.) are adjuvant therapies in case of unilateral inflammatory relapse. Therapeutic response must be evaluated precisely by clinical examination and repeated complementary investigations (laser flare photometry, multimodal imaging, perimetry, electroretinography measures).


Assuntos
Guias de Prática Clínica como Assunto , Uveíte/terapia , Corticosteroides/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Produtos Biológicos/uso terapêutico , Terapia Biológica/métodos , Prova Pericial , Humanos , Imunossupressores/uso terapêutico , Guias de Prática Clínica como Assunto/normas , Fator de Necrose Tumoral alfa/imunologia
15.
J Clin Invest ; 96(6): 2839-44, 1995 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-8675654

RESUMO

Mediterranean spotted fever due to infection by Rickettsia conorii, is characterized by a general vasculitis. This vasculitis is thought to be due to a direct injury to endothelial cells induced by R. conorii. However, production and activity of cytokines on endothelial cells is an important pathway in inflammation, and part of the underlying mechanism of vasculitis. In the present studies, human umbilical vein endothelial cells (HUVEC) infected with R. conorii actively secrete high levels of IL-8 and IL-6 (P < 0.002, and P < 0.03, respectively, compared with uninfected cells). IL-1alpha, IL-1beta, or TNFalpha were not detected in the culture supernates. Nevertheless, IL-6 and IL-8 production was due, in a large part, to a cell-associated form of IL-1 alpha expressed on R. conorii-infected HUVEC, since production of these cytokines was suppressed by 80% (P = 0.0001) and 85% (P < 0.04) by the addition of IL-1 receptor antagonist, or anti-IL-1alpha antibodies (60% inhibition, P < 0.01 and 65% inhibition, P < 0.05, respectively) and IL-1alpha was measured after lysis of R. conorii-infected HUVEC but not in uninfected cells (P < 0.01). Rickettsial lipopolysaccharide does not seem to be involved, since polymyxin B did not reduce cytokine secretion. On the contrary, infection by intracellular R. conorii appears to be necessary to induce IL-1alpha and subsequently IL-8, since formalin-fixed R. conorii did not induce cytokine production. These observations demonstrate that R. conorii-infected HUVEC secrete IL-6 and IL-8 via the induction of cell-associated IL-1alpha, providing a possible mechanism for the vasculitis observed in Mediterranean spotted fever.


Assuntos
Endotélio Vascular/imunologia , Interleucina-1/fisiologia , Interleucina-6/biossíntese , Interleucina-8/biossíntese , Rickettsia/imunologia , Anticorpos/farmacologia , Células Cultivadas , Relação Dose-Resposta a Droga , Humanos , Interleucina-1/imunologia , Interleucina-1/farmacologia , Cinética , Fatores de Tempo , Veias Umbilicais
16.
Rev Med Interne ; 27(6): 465-72, 2006 Jun.
Artigo em Francês | MEDLINE | ID: mdl-16368165

RESUMO

INTRODUCTION: Natural killer cells are cytotoxic lymphocytes of innate immunity. These last ten years our knowledge about the mechanisms that regulates NK cell function has greatly improved. Our purpose is to present a review of these new acquisitions and their potential implications in human disease. CURRENT KNOWLEDGE AND KEY POINTS: NK cell function is regulated by a repertoire of NK cell receptors and is diversified by recognition of MHC class I by a multigenic and multi-allelic family of NK receptors. Analysis of NK cell repertoire has been used to investigate features that characterize NK cells in pathological situations. Apart from their direct cytotoxic potential to eliminate target cells, recently identification of mechanisms that control NK cell mediated cytokine production and cross talk with dendritic cells emphasize the role of NK cells in the regulation of acquired immune response. FUTURE PROSPECTS AND PROJECTS: These findings have lead to a better knowledge of the importance of the NK cells in several human diseases. It has been shown that NK cells are actors of the immunosurveillance of tumoral and infectious challenges. Allo or auto reactivity of the NK cell compartment have also been suggested in autoimmune diseases, infertility or foetal loss and transplantation. Ongoing research on NK cells in the fields of human diseases is increasing and will clarify the utility of the evaluation of the NK cell compartment and their receptors in clinical practice.


Assuntos
Infecções/imunologia , Células Matadoras Naturais/fisiologia , Animais , Quimiocinas/imunologia , Citocinas/imunologia , Humanos , Vigilância Imunológica , Células Matadoras Naturais/imunologia , Modelos Animais , Neoplasias/imunologia , Receptores Imunológicos/fisiologia
17.
Rev Med Interne ; 27(10): 783-6, 2006 Oct.
Artigo em Francês | MEDLINE | ID: mdl-16978745

RESUMO

INTRODUCTION: Familial macrothrombocytopenias are a group of rare autosomal dominant platelet disorders including many syndromes in particular the May-Hegglin anomaly. They are characterized by thrombocytopenia with giant platelets and in some cases neutrophilic inclusions in peripheral blood granulocytes. Recently these different clinical entities have been demonstrated to be linked to mutations in the same gene, MYH9. CASE REPORT: We report in a young African woman presenting as a May-Hegglin anomaly a new mutation of the MYH9 gene. In regard of this case we present a brief review of the MYH9 syndrome. CONCLUSION: The MYH9 syndrome includes now several clinical entities who share some common clinical and biological characteristics such as a thrombocytopenia with giant platelets, presence or absence of other manifestations including Dohle like bodies, nephritis, sensorineural hearing loss, cataract. We report a new case in which a new mutation of the MYH9 gene was evidenced.


Assuntos
Proteínas Motores Moleculares/genética , Cadeias Pesadas de Miosina/genética , Mutação Puntual , Trombocitopenia/genética , Adenina , Adolescente , Éxons , Feminino , Humanos , Síndrome , Timina
18.
Ann Dermatol Venereol ; 133(10): 788-90, 2006 Oct.
Artigo em Francês | MEDLINE | ID: mdl-17072196

RESUMO

BACKGROUND: Splenic lymphoma with villous lymphocytes is occasionally associated with chronic hepatitis C infection. Antiviral hepatitis C therapy has been recently reported to be efficacious against splenic lymphoma with villous lymphocytes. We report a new case revealed by cutaneous symptoms. CASE REPORT: A 53-year-old patient with arthritis and neuropathy of the lower limbs consulted for vascular purpura on both legs. The blood picture showed an increase in villous lymphocytes leading to a diagnosis of splenic lymphoma with villous lymphocytes. Histologic examination of a cutaneous biopsy specimen showed thrombosis of the superficial dermal vessels, associated with cryoglobulinemia with renal and neurologic failure, a satellite of hepatitis C virus infection. The patient was treated with interferon, ribavirin and plasmapheresis. DISCUSSION: Vascular purpura, often associated with cryoglobulinemia, may reveal chronic hepatitis C infection. The efficacy of interferon and ribavirin treatment for splenic lymphoma with villous lymphocytes associated with hepatitis C infection has already been documented, and results in remission of cryoglobulinemia and lymphoma as well a eradication of viral load in 78% patients.


Assuntos
Crioglobulinemia/complicações , Hepatite C Crônica/complicações , Linfoma de Células B/complicações , Púrpura/complicações , Neoplasias Esplênicas/complicações , Humanos , Masculino , Pessoa de Meia-Idade
19.
Cancer Res ; 54(10): 2667-72, 1994 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-8168095

RESUMO

Hepatic metastasis in melanoma is affected by processes of tumor cell adhesion to sinusoidal cells, avoidance of cytotoxic cells, and local growth-promoting activity. A role for interleukin-1 (IL-1) in these events was evaluated in vitro and in vivo by specifically blocking IL-1 receptors using the naturally occurring IL-1 receptor antagonist (IL-1Ra). At 10- and 100-fold molar excess, IL-1Ra reduced the IL-1-mediated adhesion of B16 melanoma cells to cultured hepatic sinusoidal endothelial cells by 60 and 100%, respectively. Conditioned media derived from murine hepatic sinusoidal endothelial cells contain growth-promoting activity for B16 cells, and IL-1 increased its production 2.5-fold (P < 0.01). The addition of IL-1Ra to sinusoidal cells reduced the spontaneous release of the growth-promoting activity by 32% (P < 0.05). In addition, blocking IL-1 receptors on melanoma cells reduced their responsiveness to endothelial-conditioned medium (P < 0.05). A single dose of IL-1Ra (0.5 mg/kg) given to mice i.p. 2 h prior to intrasplenic injection of melanoma cells reduced the number of hepatic metastases by 50% and the metastatic volume by 70% compared to vehicle-injected controls (P < 0.01). When given 2, 4, and 6 days after the injection of tumor cells, IL-1Ra reduced the volume of metastases by 58% (P < 0.01). Fifty % of mice pretreated with 0.5 mg/kg IL-1Ra and given 3 additional doses on days 2, 4, and 6 died after 13.5 +/- 0.4 days compared to 11.3 +/- 0.2 days for controls (P < 0.01). In mice pretreated and given 10 daily doses at 5 mg/kg, there was an 80% reduction in hepatic metastases. Using this regimen, survival was 18.1 +/- 2.4 days in the IL-1Ra group and 11.2 +/- 1.5 in controls (P < 0.001). These studies demonstrate a significant role for IL-1 in implantation and growth of metastatic melanoma in the liver.


Assuntos
Neoplasias Hepáticas/secundário , Melanoma Experimental/secundário , Receptores de Interleucina-1/antagonistas & inibidores , Sialoglicoproteínas/farmacologia , Animais , Proteína Antagonista do Receptor de Interleucina 1 , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Masculino , Melanoma Experimental/mortalidade , Melanoma Experimental/patologia , Camundongos , Camundongos Endogâmicos C57BL
20.
Artigo em Francês | MEDLINE | ID: mdl-26964840

RESUMO

INTRODUCTION: Syphilis is a sexually transmitted infection (STI) related to Treponema pallidum. Secondary syphilis is the blood-borne systemic spread of Treponema. OBSERVATION: We report the case of secondary syphilis in a patient without risk of STIs factor. The clinical picture began with a genital affection followed by oral erosions and ulcers and an anterior and then posterior uveitis. Serology established the diagnosis and intravenous penicillin G treatment allowed for healing. DISCUSSION: Called the "great pretender" because of its clinical polymorphism, secondary syphilis can lead to formidable neurological and ophthalmological complications. Serological diagnosis is based on the use of treponemal and a nontreponemal tests. Penicillin G remains the treatment of choice and must be adapted according to the clinical damage.


Assuntos
Doenças da Boca/patologia , Palato Duro/patologia , Sífilis/patologia , Idoso , Feminino , Humanos , Doenças da Boca/microbiologia , Palato Duro/microbiologia , Treponema pallidum/isolamento & purificação
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