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1.
Nutr Metab Cardiovasc Dis ; 30(11): 2085-2092, 2020 10 30.
Artigo em Inglês | MEDLINE | ID: mdl-32807637

RESUMO

BACKGROUND AND AIMS: Data from animals suggest that immunoglobulins G (IgG) play a mechanistic role in atherosclerosis and diabetes through endothelial dysfunction and insulin resistance. Patients with common variable immunodeficiency (CVID), who have low circulating levels of IgG and are treated with intravenous polyclonal IgG (IVIgG), may provide an ideal model to clarify whether circulating IgG modulate endothelial function and affect insulin sensitivity in humans. METHODS AND RESULTS: We studied 24 patients with CVID and 17 matched healthy controls (HC). Endothelial function was evaluated as flow mediated dilation (FMD) of the brachial artery at baseline and 1, 7, 14, and 21 days after IVIgG infusion in the CVID patients. We measured also plasma glucose, insulin, and calculated the HOMA-IR index. We also investigated the role of human IgG on the production of Nitric Oxide (NO) in vitro in Human Coronary Artery Endothelial Cells (HCAEC). Compared to HC, FMD of CVID patients was significantly impaired at baseline (9.4 ± 0.9 and 7.6 ± 0.6% respectively, p < 0.05) but rose above normal levels 1 and 7 days after IVIgG infusion to return at baseline at 14 and 21 days. Serum insulin concentration and HOMA-IR index dropped by 50% in CVID patients after IVIgG (p < 0.002 vs. baseline). In vitro IgG stimulated NO production in HCAEC. CONCLUSIONS: Reduced IgG levels are associated with endothelial dysfunction and IVIgG stimulates endothelial function directly while improving insulin sensitivity. The current findings may suggest an anti-atherogenic role of human IgG.


Assuntos
Artéria Braquial/efeitos dos fármacos , Imunodeficiência de Variável Comum/tratamento farmacológico , Endotélio Vascular/efeitos dos fármacos , Imunoglobulina G/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Resistência à Insulina , Vasodilatação/efeitos dos fármacos , Adolescente , Biomarcadores/sangue , Glicemia/efeitos dos fármacos , Glicemia/metabolismo , Artéria Braquial/metabolismo , Artéria Braquial/fisiopatologia , Estudos de Casos e Controles , Células Cultivadas , Imunodeficiência de Variável Comum/sangue , Imunodeficiência de Variável Comum/fisiopatologia , Endotélio Vascular/metabolismo , Endotélio Vascular/fisiopatologia , Feminino , Humanos , Infusões Intravenosas , Insulina/sangue , Masculino , Óxido Nítrico/metabolismo , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
2.
Molecules ; 24(24)2019 Dec 05.
Artigo em Inglês | MEDLINE | ID: mdl-31817348

RESUMO

The aim of this study was to explore the immunomodulatory effects of the Meretrix meretrix oligopeptide (MMO, QLNWD) in cyclophosphamide (CTX)-induced immune-deficient mice. Compared to untreated, CTX-induced immune-deficient mice, the spleen and thymus indexes of mice given moderate (100 mg/kg) and high (200 mg/kg) doses of MMO were significantly higher (p < 0.05), and body weight loss was alleviated. Hematoxylin-eosin (H&E) staining revealed that MMO reduced spleen injury, thymus injury, and liver injury induced by CTX in mice. Furthermore, MMO boosted the production of immunoglobulin G (IgG) and hemolysin in the serum and promoted the proliferation and differentiation of spleen T-lymphocytes. Taken together, our findings suggest that MMO plays a vital role in protection against immunosuppression in CTX-induced immune-deficient mice and could be a potential immunomodulatory candidate for use in functional foods or immunologic adjuvants.


Assuntos
Bivalves/química , Imunodeficiência de Variável Comum , Fatores Imunológicos , Oligopeptídeos , Linfócitos T , Animais , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/imunologia , Imunodeficiência de Variável Comum/patologia , Fatores Imunológicos/química , Fatores Imunológicos/farmacologia , Masculino , Camundongos , Camundongos Endogâmicos ICR , Oligopeptídeos/química , Oligopeptídeos/farmacologia , Especificidade de Órgãos/efeitos dos fármacos , Especificidade de Órgãos/imunologia , Linfócitos T/imunologia , Linfócitos T/patologia
3.
BMJ Case Rep ; 12(3)2019 Mar 31.
Artigo em Inglês | MEDLINE | ID: mdl-30936343

RESUMO

Common variable immunodeficiency syndrome (CVID) is a heterogeneous disorder characterised by diminished levels of IgG, IgA and/or IgM, and recurrent bacterial infections. Sinopulmonary infections are most commonly reported followed by gastrointestinal (GI) infections. GI tract represents the largest immune organ with abundance of lymphoid cells, its involvement can manifest variably ranging from asymptomatic involvement to florid symptoms and signs. Diffuse nodular lymphoid hyperplasia (DNLH) of the GI tract is characterised by numerous small polypoid nodules of variable size in the small intestine, large intestine or both. It is commonly seen in association to immunodeficiency states such as CVID, IgA deficiency and chronic infections due to Giardia lamblia and Helicobacter pylori and cryptosporidiosis. Repetitive antigenic stimulation leads to lymphoid hyperplasia. We herein describe a case of DNLH of the intestine and another case of duodenal cytomegalovirus (CMV) infection associated with CVID.


Assuntos
Imunodeficiência de Variável Comum/virologia , Infecções por Citomegalovirus/complicações , Diarreia/virologia , Duodeno/patologia , Hiperplasia/virologia , Intestino Delgado/patologia , Transtornos Linfoproliferativos/virologia , Adulto , Antivirais/uso terapêutico , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/fisiopatologia , Infecções por Citomegalovirus/fisiopatologia , Duodeno/virologia , Endoscopia do Sistema Digestório , Ganciclovir/uso terapêutico , Humanos , Hiperplasia/tratamento farmacológico , Hiperplasia/fisiopatologia , Imunoglobulinas Intravenosas/uso terapêutico , Intestino Delgado/virologia , Transtornos Linfoproliferativos/tratamento farmacológico , Transtornos Linfoproliferativos/fisiopatologia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
4.
Int J Immunopathol Pharmacol ; 33: 2058738419843381, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30968712

RESUMO

Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency diseases characterized by hypogammaglobulinemia and dysfunctional immune response to invading pathogens. Previous studies have indicated that CVID is associated with microbial translocation and systemic myeloid cell activation. The goal of this study was to determine whether patients with CVID display elevated systemic levels of markers of granulocyte activation and whether the levels are further influenced by intravenous immunoglobulin (IVIg) infusions. The plasma levels of granulocyte activation markers elastase and myeloperoxidase were determined using enzyme-linked immunosorbent assay (ELISA) in 46 CVID patients and 44 healthy controls. All CVID patients were in a stable state with no apparent acute infection. In addition, granulocyte activation markers' plasma levels in 24 CVID patients were determined prior to and 1 h following IVIg administration. Neutrophil elastase and myeloperoxidase plasma levels were significantly higher in CVID patients than in healthy controls. Systemic elastase levels were further increased following IVIg administration. In vitro stimulation of 13 CVID patients' whole blood using IVIg in a therapeutically relevant dose for 2 h resulted in a significant increase in plasma elastase levels compared to unstimulated blood. The data presented here indicate that CVID is associated with chronic granulocytic activation which is further exacerbated by administering IVIg. Increased myeloperoxidase and elastase levels may contribute to associated comorbidities in CVID patients.


Assuntos
Imunodeficiência de Variável Comum/sangue , Imunodeficiência de Variável Comum/diagnóstico , Elastase de Leucócito/sangue , Peroxidase/sangue , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Imunodeficiência de Variável Comum/tratamento farmacológico , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Masculino , Pessoa de Meia-Idade , Adulto Jovem
5.
Sci Rep ; 9(1): 167, 2019 01 17.
Artigo em Inglês | MEDLINE | ID: mdl-30655568

RESUMO

Common variable immunodeficiency (CVID) patients have reduced gut microbial diversity compared to healthy controls. The reduced diversity is associated with gut leakage, increased systemic inflammation and ten "key" bacteria that capture the gut dysbiosis (dysbiosis index) in CVID. Rifaximin is a broad-spectrum non-absorbable antibiotic known to reduce gut leakage (lipopolysaccharides, LPS) in liver disease. In this study, we explored as a 'proof of concept' that altering gut microbial composition could reduce systemic inflammation, using CVID as a disease model. Forty adult CVID patients were randomized, (1:1) to twice-daily oral rifaximin 550 mg versus no treatment for 2 weeks in an open-label, single-centre study. Primary endpoints were reduction in plasma/serum levels of soluble (s) CD14, sCD25, sCD163, neopterin, CRP, TNF, LPS and selected cytokines measured at 0, 2 and 8 weeks. Secondary endpoint was changes in intra-individual bacterial diversity in stool samples. Rifaximin-use did not significantly change any of the inflammation or gut leakage markers, but decreased gut microbial diversity compared with no treatment (p = 0.002). Importantly, the gut bacteria in the CVID dysbiosis index were not changed by rifaximin. The results suggest that modulating gut microbiota by rifaximin is not the chosen intervention to affect systemic inflammation, at least not in CVID.


Assuntos
Biomarcadores/análise , Imunodeficiência de Variável Comum/tratamento farmacológico , Disbiose/tratamento farmacológico , Microbioma Gastrointestinal/efeitos dos fármacos , Inflamação/tratamento farmacológico , Rifaximina/uso terapêutico , Adolescente , Adulto , Idoso , Antibacterianos/uso terapêutico , Feminino , Fármacos Gastrointestinais/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Estudo de Prova de Conceito , Estudos Prospectivos , Adulto Jovem
6.
Blood Transfus ; 17(2): 103-111, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30036181

RESUMO

BACKGROUND: Intravenous immunoglobulin (IVIg) treatment partially replaces antibody defects and modulates innate and adaptive immune cells in patients with primary antibody deficiencies. MATERIALS AND METHODS: This study was focused on the evaluation of the effects of in vivo IVIg administration on neutrophils from patients with common variable immune disorders (CVID). We examined polymorphonuclear neutrophil (PMN) phagocytosis, PMN oxidative burst, release of neutrophil elastase, serum level of interleukin-8 and PMN expression of CXCR1, CD11c and CD66b. RESULTS: CVID patients on chronic IVIg treatment had reduced elastase release, but normal expression of CXCR1, CD66b and CD11c receptors on PMN, normal phagocytic ability and normal secretion of interleukin-8. We found that IVIg infusions rapidly reduced the serum level of interleukin-8, the expression of its receptor, CXCR1, and the release of neutrophil elastase, suggesting that IVIg exert a dampening effect on neutrophil activity. In contrast, IVIg infusions did not alter neutrophil phagocytosis or the expression of the other receptors analysed. DISCUSSION: These findings add further information regarding the anti-inflammatory role of immunoglobulins and suggest additional benefits in keeping with recent attempts to use new therapies targeting neutrophil inflammation.


Assuntos
Imunodeficiência de Variável Comum/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Interleucina-8/imunologia , Elastase de Leucócito/imunologia , Neutrófilos/efeitos dos fármacos , Adulto , Idoso , Antígenos CD/imunologia , Antígeno CD11c/imunologia , Moléculas de Adesão Celular/imunologia , Imunodeficiência de Variável Comum/sangue , Imunodeficiência de Variável Comum/imunologia , Feminino , Proteínas Ligadas por GPI/imunologia , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Interleucina-8/sangue , Masculino , Pessoa de Meia-Idade , Neutrófilos/imunologia , Fagocitose/efeitos dos fármacos , Receptores de Interleucina-8A/imunologia
7.
J Matern Fetal Neonatal Med ; 32(18): 3092-3096, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29614902

RESUMO

Immunoglobulin replacement therapy, including intravenous immunoglobulin (IVIG), is essential for pregnant women with common variable immunodeficiency (CVID) since it prevents infection and improves the health of the newborn. There are no established IVIG treatment protocols for pregnant women with CVID, and the relationship between IVIG treatment and maternal serum IgG changes during pregnancy remains unclear. Therefore, we reviewed the medical charts of four CVID patients, including one receiving subcutaneous immunoglobulin (SCIG), for IVIG dose and frequency, maternal serum IgG changes, obstetrical findings, and perinatal outcomes. There were no serious infections but one abortion and all patients continued therapy without IVIG-related adverse events. All eight children born to the patients were healthy at one month. However, the IVIG efficiency in those with CVID significantly decreased with progression of the gestational period, suggesting that IVIG dose and frequency may be changed during pregnancy to maintain stable serum IgG trough levels in women with CVID.


Assuntos
Imunodeficiência de Variável Comum/tratamento farmacológico , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Complicações na Gravidez/tratamento farmacológico , Administração Intravenosa , Adolescente , Adulto , Imunodeficiência de Variável Comum/sangue , Feminino , Humanos , Imunização Passiva/métodos , Imunoglobulina G/sangue , Gravidez , Complicações na Gravidez/sangue , Estudos Retrospectivos , Adulto Jovem
8.
Ocul Immunol Inflamm ; 27(7): 1124-1126, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30142001

RESUMO

Purpose: To describe a case of granulomatous anterior uveitis and histologically confirmed chronic granulomatous conjunctivitis in the presence of common variable immune deficiency (CVID). Methods: Interventional case report. Results: A 72-year-old female with a history of CVID treated with regular intravenous immunoglobulin (IVIG) infusions developed chronic conjunctivitis and granulomatous anterior uveitis. She responded to topical steroids, but there was recurrence upon cessation of steroid therapy. Conjunctival biopsy demonstrated micro-granulomas in the stroma and epithelium. Treatment with IVIG was maintained throughout. Conclusion: Although rare, a diagnosis of CVID should be considered in patients with recurrent conjunctivitis and uveitis of unknown etiology, especially if there is a clinical history suggestive of defective immunity. They tend to respond well to continued steroid therapy, and IVIG therapy should not be stopped.


Assuntos
Imunodeficiência de Variável Comum/complicações , Granuloma/etiologia , Uveíte Anterior/etiologia , Idoso , Biópsia , Imunodeficiência de Variável Comum/tratamento farmacológico , Túnica Conjuntiva/patologia , Feminino , Granuloma/diagnóstico , Granuloma/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Recidiva , Microscopia com Lâmpada de Fenda , Uveíte Anterior/diagnóstico , Uveíte Anterior/tratamento farmacológico
12.
J Clin Immunol ; 38(8): 864-875, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30415311

RESUMO

Many patients with primary immunodeficiency (PID) require immunoglobulin G (IgG) replacement therapy, delivered as intravenous IgG (IVIG) or subcutaneous IgG (SCIG). We aim to identify trends in efficacy and safety that would not be evident in individual studies of small patient numbers. Seven open-label, Phase 3, prospective, multicenter studies of the efficacy and safety of Hizentra® (a SCIG), conducted in Japan, Europe, and the US were summarized. Overall, 125 unique patients received 15,013 weekly infusions during a total observation period of 250.9 patient-years. Mean weekly doses of Hizentra® were 83.22-221.3 mg/kg body weight; infusion rates per patient (total body rate) were 25.2-49.3 mL/h across studies. The rates of infections and serious bacterial infections were 3.10 and 0.03 events per patient/year, respectively. Annualized rates of days hospitalized due to infection, out of work/school, and prophylactic antibiotic use were 0.95, 5.14, and 36.78 per patient, respectively. For the equivalent monthly dose, weekly Hizentra® SCIG administration resulted in expectedly-increased serum IgG trough levels in patients switching from IVIG, and maintained levels in patients switching from previous SCIG. Adverse events (AEs) totaled 5039 (events/infusion 0.094-0.773), almost all of which were mild/moderate. Three thousand one hundred ninety-seven were considered treatment-related, the most common of which were injection site reactions (2919 events; 0.001-0.592 AEs per infusion). Systemic AEs were very uncommon. The results from these seven studies indicate that Hizentra® therapy was both efficacious and well tolerated during long-term treatment. This is particularly important in patients with PID, who may require lifelong IgG replacement therapy.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Imunodeficiência de Variável Comum/tratamento farmacológico , Doenças Genéticas Ligadas ao Cromossomo X/tratamento farmacológico , Imunoglobulina G/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Ensaios Clínicos Fase III como Assunto , Europa (Continente) , Humanos , Infusões Subcutâneas , Japão , Fatores de Tempo , Estados Unidos
14.
Clin Drug Investig ; 38(10): 955-965, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30191508

RESUMO

BACKGROUND: In Italy, there is scarce evidence on the epidemiological and economic burden induced by primary antibody deficiencies. OBJECTIVE: The aim of this study was to elaborate the available epidemiological and cost data in order to estimate the annual expenditure induced by the management of patients affected by the common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) requiring immunoglobulin (Ig) replacement therapy. METHODS: A probabilistic cost-of-illness model was developed to estimate the number of patients with CVID and XLA, and the economic burden associated with their therapy in terms of direct or indirect costs. A systematic literature review was carried out to reveal both epidemiological and economic data. Furthermore, a probabilistic sensitivity analysis with 5000 Monte Carlo simulations was performed. RESULTS: The epidemiological model allowed us to estimate the number of prevalent patients affected by XLA and CVID in Italy in 2017, corresponding to 1885 (95% confidence interval [CI] 944-3145) and 133 (95% CI 115-152) patients, respectively. The estimated total expenditure for the treatment and management of patients with CVID and XLA requiring Ig replacement therapy amounts to €42.68 million (95% CI €14.38-€86.1 million). CONCLUSIONS: This information provides a comprehensive perspective of the economic issues, and facilitates better-informed public health decision making, in the management of CVID and XLA in Italy.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Imunodeficiência de Variável Comum/tratamento farmacológico , Efeitos Psicossociais da Doença , Doenças Genéticas Ligadas ao Cromossomo X/tratamento farmacológico , Imunoglobulinas/administração & dosagem , Adolescente , Adulto , Agamaglobulinemia/economia , Agamaglobulinemia/epidemiologia , Imunodeficiência de Variável Comum/economia , Imunodeficiência de Variável Comum/epidemiologia , Feminino , Doenças Genéticas Ligadas ao Cromossomo X/economia , Doenças Genéticas Ligadas ao Cromossomo X/epidemiologia , Humanos , Imunoglobulinas/economia , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Método de Monte Carlo , Prevalência , Adulto Jovem
16.
BMJ Case Rep ; 20182018 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-29559491

RESUMO

Common variable immunodeficiency (CVID) refers to a group of disorders where differentiation and maturation of B cells into plasma cells are affected, leading to decreased or defective immunoglobulin production and subsequent immunodeficiency. Symptoms may present at any age between 5 and 72 years, although more severe forms often manifest earlier in life. Milder forms may not be detected. We present an intriguing case of a 69-year-old man presenting with recurrent pneumonia caused by a rare organism Staphylococcus lugdunensis, eventually determined to be caused by CVID. The patient had a good clinical outcome after receiving immunoglobulin replacement therapy.


Assuntos
Imunodeficiência de Variável Comum/diagnóstico , Pneumonia Estafilocócica/etiologia , Idade de Início , Idoso , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/tratamento farmacológico , Farmacorresistência Bacteriana , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Pulmão/diagnóstico por imagem , Masculino , Pneumonia Estafilocócica/sangue , Recidiva , Staphylococcus lugdunensis/isolamento & purificação , Vancomicina/uso terapêutico
17.
Int J Mol Sci ; 19(2)2018 Feb 02.
Artigo em Inglês | MEDLINE | ID: mdl-29393912

RESUMO

Common variable immunodeficiency (CVID) is an immunodeficiency disorder with a high incidence of gastrointestinal manifestations and an increased risk of gastric carcinoma and lymphoma. This review discusses the latest advancements into the immunological, clinical and diagnostic aspects of gastric malignancies in patients with CVID. The exact molecular pathways underlying the relationships between CVID and gastric malignancies remain poorly understood. These include genetics, immune dysregulation and chronic infections by Helicobacter pylori. Further studies are needed to better stratify the risk for cancer in these patients, to elaborate surveillance programs aimed at preventing these complications, and to develop new and more effective therapeutic approaches.


Assuntos
Adenocarcinoma/patologia , Imunodeficiência de Variável Comum/patologia , Imunoglobulinas/deficiência , Linfoma/patologia , Neoplasias Gástricas/patologia , Adenocarcinoma/diagnóstico , Adenocarcinoma/tratamento farmacológico , Adenocarcinoma/imunologia , Corticosteroides/uso terapêutico , Antineoplásicos/uso terapêutico , Linfócitos B/efeitos dos fármacos , Linfócitos B/imunologia , Linfócitos B/patologia , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/imunologia , Árvores de Decisões , Gerenciamento Clínico , Detecção Precoce de Câncer , Helicobacter pylori/patogenicidade , Helicobacter pylori/fisiologia , Humanos , Imunoglobulinas/uso terapêutico , Linfoma/diagnóstico , Linfoma/tratamento farmacológico , Linfoma/imunologia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/imunologia , Linfócitos T/efeitos dos fármacos , Linfócitos T/imunologia , Linfócitos T/patologia
19.
Curr Probl Diagn Radiol ; 47(4): 282-284, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28583689

RESUMO

Common variable immunodeficiency is the most common primary immunodeficiency and consists of impaired immunoglobulin production causing recurrent sinopulmonary infections. The most common cause of mortality for this disorder, however, is from the development of malignancy and autoimmune disorders. One common entity that develops is a systemic granulomatous and lymphoproliferative disorder that can cause an interstitial lung disease more formally referred to as granulomatous-lymphocytic interstitial lung disease (GL-ILD). We discuss a case of a 25-year-old woman with common variable immunodeficiency and GL-ILD and review the literature to summarize the most common radiological findings to raise the suspicion for GL-ILD on high-resolution computed tomography and delineate this from infection and other mimickers. We will also review key histopathological characteristics for diagnosis and the clinical approach and treatment options for this rare disease.


Assuntos
Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico por imagem , Imunodeficiência de Variável Comum/tratamento farmacológico , Granuloma do Sistema Respiratório/diagnóstico por imagem , Granuloma do Sistema Respiratório/etiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Tomografia Computadorizada por Raios X/métodos , Adulto , Biópsia , Diagnóstico Diferencial , Feminino , Granuloma do Sistema Respiratório/tratamento farmacológico , Humanos , Doenças Pulmonares Intersticiais/tratamento farmacológico
20.
J Allergy Clin Immunol Pract ; 6(1): 159-168.e3, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28734862

RESUMO

BACKGROUND: Patients with common variable immunodeficiency (CVID) suffer frequent respiratory tract infections despite immunoglobulin replacement and are prescribed significant quantities of antibiotics. The clinical and microbiological nature of these exacerbations, the symptomatic triggers to take antibiotics, and the response to treatment have not been previously investigated. OBJECTIVES: To describe the nature, frequency, treatment, and clinical course of respiratory tract exacerbations in patients with CVID and to describe pathogens isolated during respiratory tract exacerbations. METHODS: We performed a prospective diary card exercise in 69 patients with CVID recruited from a primary immunodeficiency clinic in the United Kingdom, generating 6210 days of symptom data. We collected microbiology (sputum microscopy and culture, atypical bacterial PCR, and mycobacterial culture) and virology (nasopharyngeal swab multiplex PCR) samples from symptomatic patients with CVID. RESULTS: There were 170 symptomatic exacerbations and 76 exacerbations treated by antibiotics. The strongest symptomatic predictors for commencing antibiotics were cough, shortness of breath, and purulent sputum. There was a median delay of 5 days from the onset of symptoms to commencing antibiotics. Episodes characterized by purulent sputum responded more quickly to antibiotics, whereas sore throat and upper respiratory tract symptoms responded less quickly. A pathogenic virus was isolated in 56% of respiratory exacerbations and a potentially pathogenic bacteria in 33%. CONCLUSIONS: Patients with CVID delay and avoid treatment of symptomatic respiratory exacerbations, which could result in structural lung damage. However, viruses are commonly represented and illnesses dominated by upper respiratory tract symptoms respond poorly to antibiotics, suggesting that antibiotic usage could be better targeted.


Assuntos
Infecções Bacterianas/epidemiologia , Imunodeficiência de Variável Comum/epidemiologia , Nasofaringe/virologia , Infecções Respiratórias/epidemiologia , Viroses/epidemiologia , Adulto , Idoso , Antibacterianos/uso terapêutico , Infecções Bacterianas/diagnóstico , Infecções Bacterianas/tratamento farmacológico , Estudos de Coortes , Imunodeficiência de Variável Comum/tratamento farmacológico , Uso de Medicamentos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Estudos Prospectivos , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/tratamento farmacológico , Escarro/microbiologia , Reino Unido/epidemiologia , Viroses/diagnóstico , Viroses/tratamento farmacológico
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