Arquivos de Asma, Alergia e Imunologia
https://www.aaai-asbai.org.br/article/doi/10.5935/2318-5015.20150005
Arquivos de Asma, Alergia e Imunologia
Relato de Caso

Deficiência do componente C5 do complemento associada a meningites meningocócicas

Complement component C5 deficiency associated with meningococcal meningitis

Wilma C. Neves Forte; Tainá Mosca; Vitor A. P. Mazon; Elias J. E. Ghosn; Raphael J. P. Fins; Carlos A. Longui; Maria da Conceição S. de Menezes

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Resumo

O presente estudo teve como objetivo relatar o caso de paciente com infecções meningocócicas graves, avaliar seu sistema complemento e o de 22 membros de sua família. Foram quantificados o complemento total (CH50) e os componentes C3, C4 e C5 do complemento. Foi observado que a paciente e quatro familiares apresentavam deficiência do componente C5: dois haviam apresentado meningites meningocócicas graves e um apresentou a infecção logo após o diagnóstico da deficiência. Concluímos que a paciente com história de infecções meningocócicas graves apresentou deficiência do componente C5 do complemento, assim como quatro de seus familiares. Nossos resultados indicam que a avaliação do sistema complemento em portadores de infecções meningocócicas e em familiares próximos deva ser realizada para identificar pacientes de risco.

Palavras-chave

Immunodeficiency, complement C5, meningococcal meningitis, Neisseria.

Abstract

The present study reports the case of a patient with serious meningococcal infections and describes the results of complement system evaluation in the patient and in 22 family members. Total complement activity (CH50) and complement components C3, C4, and C5 were quantified. The patient and four family members showed C5 complement deficiency: two had developed severe meningococcal meningitis and one had the infection soon after the diagnosis of complement deficiency was made. We concluded that our patient with a history of severe meningococcal infections presented complement C5 deficiency, as did four of her family members. These findings indicate that evaluation of the complement system in patients with meningococcal infections and their family members should be performed to identify patients at risk.

Keywords

Imunodeficiência, complemento C5, meningite meningocócica, Neisseria.

Referências

1. Notarangelo LD. Primary Immunodeficiencies. J Allergy Clin Immunol. 2010;125:S182-94.

2. Brazilian Group for immunodeficiency. [site na internet]. Disponível:http://imunopediatria.org.br/. Acessado: 30 de outubro de 2014.

3. The European Society for Immunodeficiencies registry. [site na internet]. Disponível: http://esid.org/Working-Parties/Registry/New-ESID-Registry. Acessado: 30 de outubro de 2014.

4. Grumach AS, Kirschfink M. Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach. Mol Immunol. 2014;61:110-7.

5. Lima Filho AB, Silva AMR, Moura P, Sarinho ESC. Meningite meningocócica, lecitina ligadora de manose, deficiência do complemento. Rev bras alerg imunopatol. 2011;34:3-6.

6. Iturry-Yamamoto GR, Portinho CP. Sistema complemento: ativação, regulação e deficiências congênitas e adquiridas. Rev Assoc Med Bras. 2001;47:41-51.

7. Figueroa JE, Densen P. Infectious diseases associated with complement deficiencies. Clin Microbiol Rev. 1991;4:359-95.

8. Aguilar-Ramirez P. Reis ES, Florido MPC, Barbosa AS, Farah CS, CostaCarvalho BT, et al. Skipping of exon 30 in C5 gene results in complete human C5 deficiency and demonstrates the importance of C5d and CUB domains for stability. Mol Immunol. 2009;46:2116‑23.

9. Rosa DD, Pasqualotto AC, Quadros M, Prezzi SH. Deficiency of the eighth component of complement associated with recurrent meningococcal meningitis - case report and literature review. Braz J Infect Dis. 2004;8:328-30.

10. Forte WCN. Imunologia do básico ao aplicado. 3ª ed. São Paulo: Editora Atheneu; 2015. 339p.

11. Daniel DS, Dai G, Singh CR, Lindsey DR, Smith AK, Dhandayuthapani S, et al. The reduced bactericidal function of complement C5-deficient murine macrophages is associated with defects in the synthesis and delivery of reactive oxygen radicals to mycobacterial phagosomes. J Immunol. 2006; 177(7):4688-98.

12. Sáfadi MA, Bettinger JA, Maturana GM, Enwere G, Borrow R, Global Meniingococcal Initiative. Evolving meningococcal immunization strategies. Expert Rev Vaccines. 2015;14:505-17.

13. Sáfadi MAP. Perspectivas na prevenção da doença meningocócica no Brasil. Rev Imunizações (Soc Bras Imunizações). 2014;7:8-11.

14. Schejbel L, Fadnes D, Permin H, Lappegard KT, Garred P, Mollnes TE. Primary complement C5 deficiencies - molecular characterization and clinical review of two families. Immunobiology. 2013;218(10):1304‑10.

15. Delgado-Servino E, Fontán G, Lopez-Trascasa M. C5 complement deficiency in a Spanish family. Molecular characterization of the double mutation responsible for the defect. Mol Immunol. 2005;42:105-11.


Submetido em:
18/06/2015

Aceito em:
21/11/2015

6a610656a9539526272607f2 1776695468 Articles
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