Impacto do tratamento com imunoglobulina humana intravenosa no número de pneumonias em pacientes com deficiência de anticorpo
Efficacy of immunoglobulin therapy in reducing pneumonia in patients with antibody deficiencies
Fernanda A. Nobre; Isabela G. S. Gonzalez; Karina M. Melo; Ellen O. Dantas; Tatiana C. Lawrence; Victor Nudelman; Beatriz T. Costa-Carvalho
Resumo
A imunodeficiência comum variável (ICV) e a agamaglobulinemia ligada ao X (ALX) são imunodeficiências primárias caracterizadas por deficiência de anticorpos e susceptibilidade aumentada a infecções, sendo a pneumonia a infecção mais frequente. Esses pacientes são tratados com infusões regulares de imunoglobulina intravenosa (IGIV).
Objetivo: Avaliar a frequência de pneumonias e o impacto do tratamento com IGIV em 25 pacientes com ICV ou ALX. Métodos: Análise retrospectiva dos prontuários médicos de pacientes com diagnóstico confirmado de ICV ou ALX, em tratamento regular com IGIV.
Resultados: Analisamos 25 pacientes (18 com ICV e 7 com ALX; 14 homens e 11 mulheres; média de idade atual de 18 anos). A média de idade ao início dos sintomas foi de 5,7 anos e a média de idade ao diagnóstico foi de 11,5 anos. Treze pacientes (52%) apresentaram pneumonia como primeira manifestação da imunodeficiência. A pneumonia foi a infecção mais frequente nesses pacientes antes do diagnóstico – 22 pacientes (88%) tiveram pelo menos um episódio de pneumonia antes do diagnóstico e, desses, 59% tiveram mais do que 5 episódios – antes do diagnóstico houve uma média de 6,2 episódios de pneumonia por paciente. Após início do tratamento com IGIV, 10 pacientes (40%) tiveram algum episódio de pneumonia, com uma média de 1,5 episódio por paciente. Nove pacientes (36%) apresentavam algum tipo de sequela pulmonar antes do diagnóstico da imunodeficiência.
Conclusão: O tratamento com IGIV é eficaz na redução dos episódios de pneumonia nesses pacientes. O diagnóstico e tratamento precoces são de extrema importância.
Palavras-chave
Abstract
Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are primary immune disorders characterized by antibody deficiency and increased susceptibility to infections, especially pneumonia. Replacement therapy with intravenous immunoglobulin (IVIG) is the standard treatment.
Objective: To assess the frequency of pneumonia in 25 patients with CVID or XLA and the effectiveness of intravenous immunoglobulin replacement therapy in these patients.
Methods: a descriptive study, based on a retrospective analysis of medical records from 25 patients with diagnosis of CVID or XLA treated with IVIG. Results: A total of 25 patients were studied (18 with CVID and 7 with XLA; 14 male and 11 female; mean current age, 18 years). The mean age of onset of symptoms was 5.7 years and the mean age of diagnosis was 11.5 years. Pneumonia was the most common primary clinical presentation (13 patients – 52%). Twenty-two (88%) of the 25 patients had pneumonia at least once before diagnosis and 13 out of 22 of these patients had more than 5 episodes. Pre-diagnosis patients had an average of 6.2 episodes of pneumonia. After treatment with IVIG, ten patients (40%) had pneumonia – with an average of 1.5 episode of pneumonia per patient.
Conclusion: IVIG treatment provides a remarkable reduction in pneumonia in these patients. Early diagnosis and treatment is mandatory for a positive prognosis.
Keywords
Referências
1. Notarangelo LD, Fischer A, Geha RS, Casanova JL, Chapel H, Conley ME, et al. Primary immunodeficiencies: 2009 update. J Allergy Clin Immunol 2009;124:1161-78.
2. Winkelstein JA, Marino MC, Lederman HM, Jones SM, Sllivan K. X-linked agammaglobulinemia – report on a United States Registry of 201 patients. Medicine 2006;85:193-202.
3. Errante PR, Condino-Neto A. Imunodeficiência Comum Variável: revisão da literatura. Rev bras alerg imunopatol 2008;31(1):10‑18.
4. Costa-Carvalho BT, Wandalsen GF, Pulici G, Aranda CS, Solé D. Pulmonary complications in patients with antibody deficiency. Allergol Immunopathol (Madr) 2011;39:128-32.
5. Orange JS, Grossman WJ, Navickis RJ, Wilkes MM. Impact of trough IgG on pneumonia incidence in primary immunodeficiency – a metaanalysis of clinical studies. Clin Immunol 2010;137:21‑30.
6. Busse PJ, Razvi S, Cunningham-Rundles C. Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency. J Allergy Clin Immunol 2002;109:1001-4.
7. Berger M. Principles of and advances in immunoglobulin replacement therapy for primary immunodeficiency. Immunol Allergy Clin North Am 2008;28(2):413-37.
8. Maarschalk-Ellerbroek LJ, Hoepelman IM, Ellerbroek PM. Immunoglobulin treatment in primary antibody deficiency. Int J Antimicrob Agents 2011;37(5):396-404.
9. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol 1999;93:190-97.
10. Je f frey Modell Foundation. Economic Impact Study: comparing undiagnosed and diagnosed patients with primary immunodeficiencies.2007. Disponível em: www.info4pi.org.
11. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol 1999;92:34-48.
12. Plebani A, Soresina A, Rondelli R, Amato GM, Azzari C. Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian Multicenter Study. Clin Immunol 2002;104(3):221-30.
13. Aghamohammadi A, Allahverdi A, Abolhassani H, Moazzami K, Alizadeh H, et al. Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia. Respirology 2010;15:289-95.
14. Costa-Carvalho BT, Condino-Neto A, Solé D, Rosário-Filho N et al. I Consenso brasileiro sobre o uso de Imunoglobulina Humana em pacientes com imunodeficiência primária. Rev bras alerg imunopatol 2010;33:104-16.
15. Touw CM, van de Ven AA, de Jong PA, Terheggen-Lagro S, Beek E, et al. Detection of pulmonary complications in common variable immunodeficiency. Pediatr Allergy Immunol 2010;21(5):793‑805.
16. Lucas M, Lee M, Lortan J, Lopez-Granados E, Misbah S. Infections outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol 2010;125:1354-60.
17. INFO4PI.ORG – Primary Immunodeficiency Resource Center. 2011. Disponível em: http://www.info4pi.org.
Submetido em:
02/08/2011
Aceito em:
13/01/2012
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