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1.
Orv Hetil ; 160(38): 1487-1494, 2019 Sep.
Artigo em Húngaro | MEDLINE | ID: mdl-31537095

RESUMO

Immune status was investigated in 186 patients with chronic lymphoid leukaemia between January 2012 and March 2015. Incidences of infections and mortality were analysed in patients who did not receive prophylactic immunoglobulin therapy. Immunoglobulin G (IgG) levels were normal (7-17.8 g/L) or decreased in 62.37% and 35.48% of patients, respectively. We measured high immunoglobulin levels only in a few cases (2.15%). Immunoglobulin levels became increasingly lower in more advanced disease stages (Rai stages). The number of infections was inversely proportional to that. Hypogammaglobulinaemia proved to be more important than disease progression in terms of the development of infections. The most common infections were upper respiratory tract (33.07%) and sepsis (18.90%). Two months after chemotherapy, initially normal immunoglobulin levels decreased by an average of 21%, and at the same time the incidence of infections increased. The most common cause of death was sepsis: 30% occurred at low immunoglobulin levels, while 20% at normal immunoglobulin levels. According to literature, prophylactic immunoglobulin treatment is indicated in patients with chronic lymphoid leukaemia and immunodeficiency for decreasing both morbidity and mortality. According to recommendations in literature, replacement treatment must be administered in severe or moderately severe recurrent bacterial infections. Immunoglobulin prophylaxis may be provided as low dose (10 g), fix dose (18 g) or individually customized higher dose (300-400 mg/kg body weight) treatment. According to recommendations, higher dose immunoglobulin prophylaxis, administered every three weeks on six occasions, is more efficient when customized. With this dose, infection-free condition may be achieved in 50% of patients. Orv Hetil. 2019; 160(38): 1487-1494.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Agamaglobulinemia/mortalidade , Imunoglobulinas Intravenosas/uso terapêutico , Imunoglobulinas/administração & dosagem , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Sepse/mortalidade , Agamaglobulinemia/complicações , Relação Dose-Resposta a Droga , Feminino , Humanos , Hungria/epidemiologia , Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/administração & dosagem , Controle de Infecções , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/mortalidade , Masculino , Sepse/diagnóstico , Resultado do Tratamento
2.
BMC Cancer ; 19(1): 762, 2019 Aug 02.
Artigo em Inglês | MEDLINE | ID: mdl-31375083

RESUMO

BACKGROUND: Acquired immunodeficiency associated with thymoma is a rare disorder. Here we reported a case of acquired immunodeficiency with thymoma, with an unusual pattern of low CD4+ count with normal gammaglobulin levels. CASE PRESENTATION: A 70-year-old man presented to the emergency room of our hospital with a high-grade fever, headache, and nausea. He had a five-year history of unresectable thymoma treatment, including several cytotoxic regimens. He had received thoracic palliative radiotherapy 2 months prior to the emergent visit. During the previous month, he had experienced multiple febrile episodes, dry cough, fatigue, weight loss, and watery diarrhea. Upon admission, he had a high-grade fever, nausea, and immobility. Physical examination revealed indistinct consciousness, neck stiffness, and oropharyngeal candidiasis. Both cerebrospinal fluid and blood cultures yielded multiple short chains of Gram-positive rods later identified as Listeria monocytogenes, so he was diagnosed with Listeria meningitis. Intravenous administration of antibiotics was initiated, and the patient fully recovered and was discharged. Additional examination found normal immunoglobulin levels. Peripheral-blood cell counts revealed low CD4+ cell count (108 CD4+ cells/µl). His CD4+ cell count remained low after discharge. CONCLUSIONS: Our findings suggest that physicians need to be aware of severe infections due to immunodeficiency with thymoma.


Assuntos
Agamaglobulinemia/complicações , Meningite por Listeria/complicações , Timoma/complicações , Neoplasias do Timo/complicações , Administração Intravenosa , Agamaglobulinemia/etiologia , Idoso , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Contagem de Linfócito CD4 , Linfócitos T CD4-Positivos/metabolismo , Humanos , Listeria monocytogenes/isolamento & purificação , Masculino , Meningite por Listeria/tratamento farmacológico , Meningite por Listeria/microbiologia , Radioterapia/efeitos adversos , Timoma/radioterapia , Neoplasias do Timo/radioterapia , Resultado do Tratamento
3.
Indian Pediatr ; 56(5): 423-425, 2019 05 15.
Artigo em Inglês | MEDLINE | ID: mdl-31102385

RESUMO

BACKGROUND: X-linked agammaglobulinemia, a primary immunodeficiency, can present with musculoskeletal manifestations. CASE CHARACTERISTICS: A 4-year-old boy, diagnosed as systemic juvenile idiopathic arthritis at the age of 3 years and treated with biological agents, presented with fever, dyspnea and chest pain. Blood culture and pericardial fluid culture revealed Achromobacter xylosoxidans. OUTCOME: Investigation revealed normal serum ferritin but low levels of serum immunoglobulins. Further immunological work-up revealed diagnosis of X-linked agammaglobulinemia. Child improved on antibiotic therapy; treatment with steroids and biological was discontinued. MESSAGE: Underlying immunodeficiency disease must be looked for in children suspected to have juvenile arthritis, more so if they develop unusual serious infection in response to immunomodulatory therapy.


Assuntos
Achromobacter denitrificans/isolamento & purificação , Agamaglobulinemia/diagnóstico , Artrite Juvenil/diagnóstico , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Infecções por Bactérias Gram-Negativas/etiologia , Sepse/etiologia , Agamaglobulinemia/complicações , Pré-Escolar , Diagnóstico Diferencial , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Infecções por Bactérias Gram-Negativas/diagnóstico , Humanos , Masculino , Sepse/diagnóstico
5.
Int J Immunopathol Pharmacol ; 33: 2058738419843364, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30968707

RESUMO

Humoral immunodeficiency with accompanying infections is an indication for human immunoglobulin replacement therapy. Whether treatment will be lifelong or necessary only temporarily depends on the nature of deficiency: primary (persistent) or secondary (persistent or transient). It is not always easy to distinguish between primary and secondary immunodeficiency, especially in adults. The article presents a case of a 39-year-old patient with anamnesis and medical tests results that suggested primary humoral immunodeficiency. The deficiency was diagnosed for the first time at the age of 38, when the patient was pregnant. The patient was qualified for immunoglobulin G replacement therapy. Clinical improvement was achieved. After the end of pregnancy, systematic improvement in immunological parameters was observed, suggesting the resolution of immunodeficiency. A decision was made to discontinue immunoglobulin replacement. Due to the ability to respond to vaccine, confirmed during diagnosis, preventive vaccines were recommended. There was no recurrence of serious infections. The clinical course finally enabled a diagnosis of secondary immunodeficiency. The presented case shows the importance of an active approach to the diagnostic and therapeutic process, constant assessment of clinical course, monitoring of IgG concentrations, and the awareness that in the situation when we do not have a genetic confirmation of the disease, the diagnosis may change.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Deficiência de IgG/tratamento farmacológico , Imunoglobulina G/administração & dosagem , /tratamento farmacológico , Adulto , Agamaglobulinemia/complicações , Agamaglobulinemia/diagnóstico , Esquema de Medicação , Feminino , Humanos , Deficiência de IgG/complicações , Deficiência de IgG/diagnóstico , /etiologia , Infusões Intravenosas/tendências , Recidiva
6.
Clin Immunol ; 200: 16-18, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30630113

RESUMO

Severe combined immunodeficiency (SCID) can be caused by deleterious mutations in DCLRE1C, leading to deficient non-homologous end joining by compromising the function of the Artemis protein. This impairs the process of V(D)J recombination of the T- and B-cell receptors and typically results in radiosensitive T-, B-, NK+ SCID presenting during the first months of life. We present a case of a 3-year-old girl with two novel compound heterozygous variants in DCLRE1C (c.58G>C and c.374A>C) that were associated with marked reduced numbers of peripheral T- and B-cells and undetectable total serum IgG. Despite the severe laboratory phenotype, the patient had a normal development, albeit failure to thrive (-2.5 to -3 SD), during her first years of life including day-care attendance at preschool for 1.5 years. After being diagnosed with pneumonia the clinical picture of SCID was recognized and the girl successfully underwent hematopoietic stem-cell transplantation.


Assuntos
Agamaglobulinemia/genética , Proteínas de Ligação a DNA/genética , Endonucleases/genética , Imunodeficiência Combinada Severa/genética , Agamaglobulinemia/complicações , Agamaglobulinemia/imunologia , Pré-Escolar , Feminino , Heterozigoto , Humanos , Mutação , Pneumonia por Pneumocystis/diagnóstico , Pneumonia por Pneumocystis/etiologia , Imunodeficiência Combinada Severa/complicações , Imunodeficiência Combinada Severa/diagnóstico , Imunodeficiência Combinada Severa/imunologia
7.
Clin Immunol ; 200: 39-42, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30690192

RESUMO

Good syndrome is an immunodeficiency presenting with thymoma, hypogammaglobulinemia and almost absent B cells. To investigate the origin of the B-cell lymphopenia in these patients, we studied B cell differentiation in the bone marrow of Good syndrome patients. We found very low numbers of precursor B cells in bone marrow of Good syndrome patients and a differentiation arrest after the pro-B-cell stage; this is different from other agammaglobulinemia patients with a defect in pre B-cell receptor signaling.


Assuntos
Agamaglobulinemia/imunologia , Linfócitos B/citologia , Medula Óssea , Linfopenia/imunologia , Linfopoese/imunologia , Células Precursoras de Linfócitos B/citologia , Timoma/imunologia , Neoplasias do Timo/imunologia , Agamaglobulinemia/complicações , Agamaglobulinemia/terapia , Idoso , Anti-Infecciosos/uso terapêutico , Linfócitos B/imunologia , Feminino , Humanos , Imunoglobulinas/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/imunologia , Síndromes de Imunodeficiência/terapia , Fatores Imunológicos/uso terapêutico , Linfopenia/complicações , Linfopenia/terapia , Masculino , Pessoa de Meia-Idade , Células Precursoras de Linfócitos B/imunologia , Síndrome , Timectomia , Timoma/complicações , Timoma/terapia , Neoplasias do Timo/complicações , Neoplasias do Timo/terapia
9.
Intern Med ; 58(9): 1341-1347, 2019 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-30568129

RESUMO

To explore non-motor comorbidities of myasthenia gravis (MG), we present two cases of thymoma-associated MG patients. Alopecia, pure red cell aplasia, and thymoma- associated multiorgan autoimmunity were observed in Case 1, and alopecia, thrombocytopenia, hypogammaglobulinemia and nephrotic syndrome were observed in Case 2. In both cases, autoreactive T lymphocytes inappropriately stimulated by thymus tissue may have played key roles in generating the various autoimmune-associated symptoms. Consequently, systemic immunological involvement due to the thymoma-associated breakdown of immunoregulations in both motor and non-motor systems should be considered in MG patients.


Assuntos
Miastenia Gravis/complicações , Timoma/complicações , Neoplasias do Timo/complicações , Agamaglobulinemia/complicações , Alopecia/complicações , Autoimunidade/fisiologia , Feminino , Humanos , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/complicações , Linfócitos T/imunologia , Trombocitopenia/complicações
10.
Immunol Allergy Clin North Am ; 39(1): 31-47, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30466771

RESUMO

Secondary hypogammaglobulinemia is a common development in patients treated with immunomodulatory agents for autoimmune, connective tissue, and malignant diseases. It has been observed in the medical management of patients undergoing hematopoietic stem cell and solid organ transplantation. Some patients have preexisting immunodeficiency associated with these illnesses; immunosuppressive treatment magnifies their immune defect. This article reviews immunosuppressive medications, including biological treatments that cause secondary hypogammaglobulinemia. It summarizes risk factors for rituximab-induced hypogammaglobulinemia, such as preexisting low immunoglobulin G levels, CD19 levels, host factors, and additive effect of all immunomodulatory drugs used. The evaluation and management of secondary hypogammaglobulinemia are discussed.


Assuntos
Agamaglobulinemia/complicações , Agamaglobulinemia/terapia , Fatores Imunológicos/efeitos adversos , Transplante de Órgãos/efeitos adversos , Agamaglobulinemia/etiologia , Terapia Biológica/efeitos adversos , Terapia Biológica/métodos , Gerenciamento Clínico , Humanos , Imunidade Humoral , Fatores Imunológicos/uso terapêutico , Imunossupressores/efeitos adversos , Neoplasias/complicações , Neoplasias/terapia , Fatores de Risco
11.
Immunol Allergy Clin North Am ; 39(1): 95-111, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30466775

RESUMO

Immunoglobulin replacement therapy is the cornerstone of management for most primary immunodeficiency disease patients. The selection of a particular product, dose, and route of administration requires an understanding of the features of therapeutic immunoglobulin as well as patient-specific risk factors in order to maximize efficacy and tolerability and minimize risk. Individualizing therapy, taking into consideration the burdens of care, is necessary in order to optimize patient outcomes.


Assuntos
Agamaglobulinemia/terapia , Medicina de Precisão , Agamaglobulinemia/complicações , Agamaglobulinemia/diagnóstico , Agamaglobulinemia/etiologia , Animais , Tomada de Decisão Clínica , Efeitos Psicossociais da Doença , Gerenciamento Clínico , Vias de Administração de Medicamentos , Acesso aos Serviços de Saúde , Humanos , Imunização Passiva/efeitos adversos , Imunização Passiva/métodos , Imunoglobulina G/administração & dosagem , Imunoglobulina G/efeitos adversos , Imunoglobulina G/uso terapêutico , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/efeitos adversos , Imunoglobulinas Intravenosas/farmacocinética , Imunoglobulinas Intravenosas/uso terapêutico , Medicina de Precisão/efeitos adversos , Medicina de Precisão/métodos , Resultado do Tratamento
12.
Intern Med J ; 49(3): 358-363, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30129248

RESUMO

BACKGROUND: Acquired hypogammaglobulinaemia secondary to haematological malignancies is associated with increased infection risk. Immunoglobulin (Ig) replacement reduces major infections but not mortality, and is costly. No prospective randomised trials have compared Ig replacement with prophylactic antibiotics. AIMS: To identify variation in current practice regarding management of secondary hypogammaglobulinaemia in Australia and New Zealand, to identify barriers to best practice, and to inform the development of a clinical trial assessing antibiotic prophylaxis in secondary hypogammaglobulinaemia. METHODS: We conducted an online survey of current clinical practice regarding management of secondary hypogammaglobulinaemia among haematologists in Australia and New Zealand. RESULTS: Seventy-two haematologists responded; 89% of whom reported commencing Ig replacement for secondary hypogammaglobulinaemia in the setting of recurrent or severe infection. Most monitored trough immunoglobulin G levels, most often 3 monthly. Criteria for stopping Ig replacement varied. Most respondents recommended influenza and pneumococcal vaccination, while only 21% reported using antibiotic prophylaxis. Few respondents (3%) reported prescribing prophylactic antibiotics before commencing Ig replacement. Most reported an interest in recruiting patients to a clinical trial comparing Ig replacement with prophylactic antibiotics. CONCLUSION: In comparison to limited international data, this survey finds variation in practice, which may be due to differences in local policies governing access to Ig. These findings highlight the need for research into the indications for Ig commencement and cessation, and will inform design of prospective trials of infection prevention in secondary hypogammaglobulinaemia.


Assuntos
Agamaglobulinemia/complicações , Antibioticoprofilaxia , Neoplasias Hematológicas/complicações , Imunoglobulinas Intravenosas/uso terapêutico , Austrália , Infecções Bacterianas/prevenção & controle , Neoplasias Hematológicas/tratamento farmacológico , Humanos , Imunoglobulina G/sangue , Nova Zelândia , Padrões de Prática Médica , Estudos Prospectivos , Inquéritos e Questionários , Vacinação , Viroses/prevenção & controle
13.
Transfusion ; 58 Suppl 3: 3056-3064, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30536429

RESUMO

Primary immunodeficiency (PID) diseases result from genetic defects of the immune system that increase a patient's susceptibility to infections. The types of infections that occur in patients with PID diseases are dictated largely by the nature of the immunodeficiency, which can be defined by dysfunction of cellular or humoral defenses. An increasing number of PID diseases, including those with both cellular and humoral defects, have antibody deficiency as a major feature, and as a result can benefit from immunoglobulin replacement therapy. In fact, the most common PID diseases worldwide are antibody deficiencies and include common variable immunodeficiency, congenital agammaglobulinemia, hyper-IgM syndrome, specific antibody deficiency, and Good syndrome. Although immunoglobulin replacement therapy is the cornerstone of treatment for the majority of these conditions, a thorough understanding of the specific infections for which these patients are at increased risk can hasten diagnosis and guide additional therapies. Moreover, the infection trends in some patients with PID disease who have profound defects of cellular immunity, such as autosomal-dominant hyper-IgE syndrome (Job/Buckley syndrome) or dedicator of cytokinesis 8 (DOCK8) deficiency, suggest that select patients might benefit from immunoglobulin replacement therapy even if their immunodeficiency is not limited to antibody defects. In this review, we provide an overview of the predisposition to infections seen in PID disease that may benefit from immunoglobulin replacement therapy.


Assuntos
Imunoglobulinas/uso terapêutico , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/terapia , /imunologia , Agamaglobulinemia/complicações , Agamaglobulinemia/imunologia , Agamaglobulinemia/terapia , Imunodeficiência de Variável Comum/imunologia , Imunodeficiência de Variável Comum/terapia , Doenças Genéticas Ligadas ao Cromossomo X/imunologia , Doenças Genéticas Ligadas ao Cromossomo X/terapia , Humanos , Síndromes de Imunodeficiência/classificação , Síndromes de Imunodeficiência/imunologia , Fatores de Risco
14.
Emerg Infect Dis ; 24(12): 2382-2386, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30457541

RESUMO

We report a disseminated infection caused by Spiroplasma apis, a honeybee pathogen, in a patient in France who had X-linked agammaglobulinemia. Identification was challenging because initial bacterial cultures and direct examination by Gram staining were negative. Unexplained sepsis in patients with agammaglobulinemia warrants specific investigation to identify fastidious bacteria such as Spiroplasma spp.


Assuntos
Agamaglobulinemia/complicações , Infecções por Bactérias Gram-Negativas/diagnóstico , Infecções por Bactérias Gram-Negativas/etiologia , Spiroplasma , Adulto , Agamaglobulinemia/diagnóstico , Agamaglobulinemia/terapia , Antibacterianos/uso terapêutico , Biópsia , França , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Infecções por Bactérias Gram-Negativas/tratamento farmacológico , Infecções por Bactérias Gram-Negativas/microbiologia , Humanos , Masculino , RNA Ribossômico 16S/metabolismo , Pele/microbiologia , Pele/patologia , Spiroplasma/classificação , Spiroplasma/genética , Resultado do Tratamento
17.
J Pediatr Endocrinol Metab ; 31(10): 1155-1159, 2018 Oct 25.
Artigo em Inglês | MEDLINE | ID: mdl-30243016

RESUMO

Background Infantile free sialic acid storage disease (ISSD) is a severe multisystemic disorder characterized by the accumulation of free sialic acid in lysosomes. Case presentation The patient presented prenatally with fetal ascites and large scrotal hernias, without pleural or pericardial effusion. During the infantile period, he was diagnosed with permanent isolated immunoglobulin G (IgG) hypogammaglobulinemia, which thus far has rarely been associated with ISSD. The analysis of the SLC17A5 gene revealed a novel homozygous 94 bp gene deletion. We further provide a detailed description of pre- and postnatal clinical and radiographic findings. Conclusions Fetal ascites could be the first sign of several lysosomal storage diseases (LSDs), including ISSD. The analysis of LSD gene panels is an effective approach to diagnosis in the case of non-specific symptoms and when specific biochemical tests are not easily available.


Assuntos
Agamaglobulinemia/complicações , Mutação , Transportadores de Ânions Orgânicos/genética , Doença do Armazenamento de Ácido Siálico/complicações , Simportadores/genética , Agamaglobulinemia/sangue , Agamaglobulinemia/diagnóstico por imagem , Agamaglobulinemia/genética , Encéfalo/diagnóstico por imagem , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Doença do Armazenamento de Ácido Siálico/sangue , Doença do Armazenamento de Ácido Siálico/diagnóstico por imagem , Doença do Armazenamento de Ácido Siálico/genética , Ultrassonografia
18.
Am J Physiol Heart Circ Physiol ; 315(5): H1358-H1367, 2018 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-30095974

RESUMO

Heart-specific antibodies have been widely associated with myocardial infarction (MI). However, it remains unclear whether autoantibodies mediate disease progression or are a byproduct of cardiac injury. To disambiguate the role of immunoglobulins in MI, we characterized the development of ischemic heart failure in agammaglobulinemic mice (AID-/-µS-/-). Although these animals can produce functional B cells, they cannot synthesize secretory IgM (µS-/-) or perform Ig class switching (AID-/-), leading to complete antibody deficiency. Agammaglobulinemia did not affect overall post-MI survival but resulted in a significant reduction in infarct size. Echocardiographic analyses showed that, compared with wild-type infarcted control mice, AID-/-µS-/- mice exhibited improved cardiac function and reduced remodeling on day 56 post-MI. These differences remained significant even after animals with matched infarct sizes were compared. Infarcted AID-/-µS-/- mice also showed reduced myocardial expression levels of transcripts known to promote adverse remodeling, such as matrix metalloproteinase-9, collagen type I a1, collagen type III a1, and IL-6. An unbiased screening of the heart reactivity potential in the plasma of wild-type MI animals revealed the presence of antibodies that target the myocardial scar and collagenase-sensitive epitopes. Moreover, we found that IgG accumulated within the scar tissues of infarcted mice and remained in close proximity with cells expressing Fcγ receptors (CD16/32), suggesting the existence of an in situ IgG-Fcγ receptor axis. Collectively, our study results confirm that antibodies contribute to ischemic heart failure progression and provide novel insights into the mechanisms underlying this phenomenon. NEW & NOTEWORTHY Our study sheds some light on the long-standing debate over the relevance of autoantibodies in heart failure and might stimulate future research in the field. The observation of extracellular matrix-specific antibodies and the detection of Fcγ receptor-expressing cells within the scar provide novel insights into the mechanisms by which antibodies may contribute to adverse remodeling.


Assuntos
Agamaglobulinemia/imunologia , Autoanticorpos/imunologia , Insuficiência Cardíaca/prevenção & controle , Switching de Imunoglobulina , Imunoglobulina M/imunologia , Infarto do Miocárdio/imunologia , Miocardite/prevenção & controle , Miocárdio/imunologia , Agamaglobulinemia/complicações , Agamaglobulinemia/genética , Agamaglobulinemia/metabolismo , Animais , Autoanticorpos/metabolismo , Modelos Animais de Doenças , Matriz Extracelular/imunologia , Matriz Extracelular/metabolismo , Matriz Extracelular/patologia , Fibrose , Insuficiência Cardíaca/imunologia , Insuficiência Cardíaca/metabolismo , Insuficiência Cardíaca/patologia , Switching de Imunoglobulina/genética , Imunoglobulina M/genética , Imunoglobulina M/metabolismo , Masculino , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Infarto do Miocárdio/complicações , Infarto do Miocárdio/metabolismo , Infarto do Miocárdio/patologia , Miocardite/imunologia , Miocardite/metabolismo , Miocardite/patologia , Miocárdio/metabolismo , Miocárdio/patologia , Receptores de IgG/imunologia , Receptores de IgG/metabolismo , Função Ventricular Esquerda , Remodelação Ventricular
19.
BMC Neurol ; 18(1): 97, 2018 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-30016937

RESUMO

BACKGROUND: Immunoglobulin associated meningitis is a rare disease that mimics infectious meningitis. This is, to our knowledge, the first case of Immunoglobulin-associated meningitis described in a patient with Systemic Lupus Erythematosus and hypogammaglobulinemia secondary to Rituximab. CASE PRESENTATION: A 46-year-old female with a past medical history of Systemic Lupus Erythematosus, presented with meningismus 36 h after first infusion of intravenous immunoglobulin. The cerebrospinal fluid analysis showed neutrophilic pleocytosis and hyperproteinorrachia. All microbiological tests were negative. The patient recovered remarkably fast without sequela after just five days of antibiotic therapy. CONCLUSION: Systemic Lupus Erythematosus is a well-documented risk factor for aseptic meningitis associated with other drugs. Possibly, it is also a risk factor for Immunoglobulin associated meningitis. This diagnosis, although rare, should be considered in patients receiving Immunoglobulin since it is a self-limited condition and treatment is supportive.


Assuntos
Agamaglobulinemia , Fatores Imunológicos/efeitos adversos , Lúpus Eritematoso Sistêmico , Meningite Asséptica , Rituximab/efeitos adversos , Agamaglobulinemia/induzido quimicamente , Agamaglobulinemia/complicações , Agamaglobulinemia/diagnóstico , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Meningite Asséptica/diagnóstico , Meningite Asséptica/etiologia , Meningite Asséptica/fisiopatologia , Pessoa de Meia-Idade , Fatores de Risco
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