RESUMO
BACKGROUND: Common variable immunodeficiency (CVID) is characterised by hypogammaglobulinaemia and a broad clinical spectrum, mainly showing recurrent bacterial infections accompanied sometimes by increased susceptibility to chronic lung disease, autoimmunity, and neoplastic diseases. OBJECTIVES: To evaluate the clinical and immunological characteristics of patients with CVID in Mexico. METHODS: This is a retrospective analysis of 43 patients with CVID from the Immunology Division of seven different reference centres in Mexico. Patients were diagnosed according to the diagnostic criteria of the European Society for Immunodeficiency Diseases. We collected demographics, clinical and immunological data from each patient and a statistical analysis was performed. RESULTS: There were 23 (53.5%) male and 20 (46.5%) female patients. Median age at onset of disease was 13.7 years, and median age at diagnosis was 19 years. Average delay in diagnosis was 12.5 years. The median total serum levels of IgG, IgM, and IgA at diagnosis were 175, 18, and 17.8mg/dL, respectively. The mean percentage of CD19+ B cells was 8.15%. Sinusitis (83%), pneumonia (83%), gastrointestinal infection (70%), and acute otitis media (49%) were the most common manifestations. Bronchiectasis was present in 51% of the patients, 44% manifested non-infectious chronic diarrhoea, and 70% experienced weight loss. Autoimmunity was present in 23% of the patients; haemolytic anaemia and autoimmune thrombocytopenic purpura were the most common presentations. Allergy was present in 30.2% of patients, with allergic rhinitis and asthma being the most frequent types. Two patients developed malignancy. All the patients received Intravenous immunoglobulin (IVIG) as a fundamental part of the treatment at a mean dose of 408mg/kg. CONCLUSION: This is the first cohort of CVID reported in Mexico We found that infection diseases were the most frequent presentations at onset. Moreover, patients had an average diagnosis delay of twelve years and thus a major prevalence of bronchiectasis. We suggest performing an extended analysis of patients with CVID patients in other Latin American countries.
Assuntos
Asma/imunologia , Linfócitos B/imunologia , Bronquiectasia/imunologia , Imunodeficiência de Variável Comum/imunologia , Infecções/imunologia , Adolescente , Adulto , Antígenos CD19/metabolismo , Autoimunidade , Criança , Estudos de Coortes , Imunodeficiência de Variável Comum/fisiopatologia , Imunodeficiência de Variável Comum/terapia , Feminino , Humanos , Imunoglobulinas/sangue , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , México , Estudos Retrospectivos , Adulto JovemRESUMO
INTRODUCTION: Common variable immunodeficiency is characterized by defective antibody production and recurrent pulmonary infections. Intravenous immunoglobulin is the treatment of choice, but the effects of Intravenous immunoglobulin on pulmonary defense mechanisms are poorly understood. OBJECTIVE: The aim of this study was to verify the impact of intravenous immunoglobulin on the physical properties of the sputum and on inflammatory alterations in the airways of patients with Common variable immunodeficiency associated with bronchiectasis. METHOD: The present study analyzed sputum physical properties, exhaled NO, inflammatory cells in the sputum, and IG titers in 7 patients with Common variable immunodeficiency and bronchiectasis with secretion, immediately before and 15 days after Intravenous immunoglobulin. A group of 6 patients with Common variable immunodeficiency and bronchiectasis but no sputum was also studied for comparison of the basal IgG level and blood count. The 13 patients were young (age=36+/-17 years) and comprised predominantly of females (n=11). RESULTS: Patients with secretion presented significantly decreased IgG and IgM levels. Intravenous immunoglobulin was associated with a significant decrease in exhaled NO (54.7 vs. 40.1 ppb, p<0.05), sputum inflammatory cell counts (28.7 vs. 14.6 cells/mm(3), p<0.05), and a significant increase in respiratory mucus transportability by cough (42.5 vs. 65.0 mm, p < 0.05). CONCLUSION: We concluded that immunoglobulin administration in Common variable immunodeficiency patients results in significant improvement in indexes of inflammation of the airways with improvement in the transportability of the respiratory mucus by cough.
Assuntos
Bronquiectasia , Imunodeficiência de Variável Comum , Imunoglobulinas Intravenosas/uso terapêutico , Depuração Mucociliar/fisiologia , Infecções Respiratórias , Escarro , Adulto , Bronquiectasia/tratamento farmacológico , Bronquiectasia/imunologia , Bronquiectasia/fisiopatologia , Contagem de Células , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/imunologia , Imunodeficiência de Variável Comum/fisiopatologia , Tosse/imunologia , Tosse/fisiopatologia , Feminino , Humanos , Imunoglobulina G/análise , Imunoglobulina M/análise , Masculino , Muco/fisiologia , Óxido Nítrico/análise , Infecções Respiratórias/tratamento farmacológico , Infecções Respiratórias/imunologia , Infecções Respiratórias/fisiopatologia , Escarro/citologia , Escarro/efeitos dos fármacos , Escarro/imunologia , Estatísticas não Paramétricas , Fatores de TempoRESUMO
INTRODUCTION: Common variable immunodeficiency is characterized by defective antibody production and recurrent pulmonary infections. Intravenous immunoglobulin is the treatment of choice, but the effects of Intravenous immunoglobulin on pulmonary defense mechanisms are poorly understood. OBJECTIVE: The aim of this study was to verify the impact of intravenous immunoglobulin on the physical properties of the sputum and on inflammatory alterations in the airways of patients with Common variable immunodeficiency associated with bronchiectasis. METHOD: The present study analyzed sputum physical properties, exhaled NO, inflammatory cells in the sputum, and IG titers in 7 patients with Common variable immunodeficiency and bronchiectasis with secretion, immediately before and 15 days after Intravenous immunoglobulin. A group of 6 patients with Common variable immunodeficiency and bronchiectasis but no sputum was also studied for comparison of the basal IgG level and blood count. The 13 patients were young (age=36±17 years) and comprised predominantly of females (n=11). RESULTS: Patients with secretion presented significantly decreased IgG and IgM levels. Intravenous immunoglobulin was associated with a significant decrease in exhaled NO (54.7 vs. 40.1 ppb, p<0.05), sputum inflammatory cell counts (28.7 vs. 14.6 cells/mm³, p<0.05), and a significant increase in respiratory mucus transportability by cough (42.5 vs. 65.0 mm, p < 0.05). CONCLUSION: We concluded that immunoglobulin administration in Common variable immunodeficiency patients results in significant improvement in indexes of inflammation of the airways with improvement in the transportability of the respiratory mucus by cough.
Assuntos
Adulto , Feminino , Humanos , Masculino , Bronquiectasia , Imunodeficiência de Variável Comum , Imunoglobulinas Intravenosas/uso terapêutico , Depuração Mucociliar/fisiologia , Infecções Respiratórias , Escarro , Bronquiectasia/tratamento farmacológico , Bronquiectasia/imunologia , Bronquiectasia/fisiopatologia , Contagem de Células , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/imunologia , Imunodeficiência de Variável Comum/fisiopatologia , Tosse/imunologia , Tosse/fisiopatologia , Imunoglobulina G/análise , Imunoglobulina M/análise , Muco/fisiologia , Óxido Nítrico/análise , Infecções Respiratórias/tratamento farmacológico , Infecções Respiratórias/imunologia , Infecções Respiratórias/fisiopatologia , Estatísticas não Paramétricas , Escarro/citologia , Escarro/efeitos dos fármacos , Escarro/imunologia , Fatores de TempoRESUMO
BACKGROUND: The primary immunodeficiency diseases cause a deficit in the production of antibodies. The chronic sinopulmonary disease is common and their clinic symptoms are diverse (pneumonia, bronchiectasis). OBJECTIVE: To know the frequency and type of pulmonary abnormalities in patients with primary immunodeficiency in treatment with intravenous immunoglobulin. MATERIAL AND METHODS: 24 files of patients with primary immunodeficiency were selected. Age, sex, primary immunodeficiency type, time of immunoglobulin treatment, chest X-ray finding, pulmonary computed tomography of high resolution (HRCT) and pulmonary function tests were registered. Measures of central tendency were calculated. RESULTS: There was no predominance of gender; the average age was 14 years old. The common variable immunodeficiency and the Bruton's hypogammaglobulinemia represented 91% of the patients. The X-ray of thorax was abnormal in 33%, although there were not bronchiectasis. The high-resolution computed tomography scan (HRCT) was abnormal in 67%, and 75% had bronchiectasis. CONCLUSIONS: The pulmonary complications are common despite therapy with intravenous immunoglobulin. The HRCT is better than X-ray in these patients.
Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/complicações , Pneumopatias/epidemiologia , Adolescente , Adulto , Agamaglobulinemia/complicações , Agamaglobulinemia/terapia , Bronquiectasia/diagnóstico por imagem , Bronquiectasia/epidemiologia , Bronquiectasia/imunologia , Bronquiectasia/fisiopatologia , Criança , Pré-Escolar , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/terapia , Suscetibilidade a Doenças , Feminino , Humanos , Síndromes de Imunodeficiência/terapia , Pneumopatias/diagnóstico por imagem , Pneumopatias/imunologia , Pneumopatias/fisiopatologia , Pneumopatias/prevenção & controle , Masculino , Prevalência , Testes de Função Respiratória , Tomografia Computadorizada por Raios X , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X/complicações , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X/terapiaRESUMO
Se presentan 2 pacientes con bronquiectasia en los cuales se demostró inmunodefiencia común variable (IDCV). Ambos pacientes fueron tratados con gammaglobulina con buena respuesta. Consideramos que el estudio de pacientes con bronquiectasias debe investigarse la IDCV
Assuntos
Humanos , Masculino , Criança , Adulto , Bronquiectasia/complicações , Imunodeficiência de Variável Comum/complicações , Bronquiectasia/imunologia , Bronquiectasia/cirurgiaRESUMO
o presente trabalho tem como objetivo a revisão dos principais tópicos relativos à bronquiectasia,desde o conceito até o tratamento,enfocando etiologia,patogenia,tipos,manifestações clinicas,diagnóstico,tratamento,complicações e prognóstico