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Regulación de la Actividad del Complemento
La dermatomiositis es una miopatía inflamatoria caracterizada por una vasculitis del endomisio mediada por el complemento. Pacientes con dermatomiositis que no responden a la terapia convencional, muestran mejoría con la aplicación de IgGIV a grandes dosis.
Las biopsias de músculo de pacientes tratados con IgGIV indican la desaparición de los depósitos de C3b y de los complejos de ataque a la membrana (C5b6-9), en los capilares del endomisio, con restablecimiento de la red capilar.
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Es probable que la IgGIV interfiera la captación de fragmentos de C3b o C4b, formando complejos entre estos fragmentos y un sitio aceptor específico en la molécula de IgG (26).
Un resumen de las acciones moduladoras negativas de las respuestas inmunes en enfermedades inflamatorias crónicas y autoinmunes, así como las dosis de la IgGIV empleadas como tratamiento en ellas se aprecian en la tabla 1.
Existen informes a cerca del beneficio recibido en más de 35 enfermedades tratadas con IgGIV, algunas de ellas descritas en la tabla 2 (27).
Tabla 2. Enfermedades con resultados benéficos luego de la terapia con IgGIV
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Enfermedades |
1. Desórdenes inmuno-hematológicos |
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2. Enfermedades autoinmunes autoinmune |
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3. Enfermedades con etiología inmunopatológica |
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Referencias Bibliográficas
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Fuentes Bibliográficas
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- 18. Lokwood CM. New treatment strategies for vasculitis: the role of intravenous immune globulin therapy. Clin Exp Immunol 1996;104 (Supl):577-582.
- 19. Fong KJ, Kaplan SL. The use of intravenous immunoglobulin in Kawasaki disease. Semin Pediatr Infect Dis 1992;3:174-178.
Lecturas Recomendadas
- 20. Shimozato T, Iwata M, Kawada H et al. Human immunoglobulin preparation for intravenous use induces elevation of cellular cyclic adenosine 3’5′-monophosphate levels, resulting in suppression of tumor necrosis factor alpha and interleukin-1 production. Immunology 1991;72:497-501.
- 21. Aukrust P, Froland SS, Liabakk NB, et al. Release of cytokines, soluble cytokine receptors, and interleukin-1 receptor antagonist after intravenous immunoglobulin administration in vivo. Blood 1994;84:2136-2143.
- 22. Leung DYM. Kawasaki syndrome: immunomodulatory benefit and potential toxin neutralization by intravenous immune globulin. Clin Exp Immunol 1996;104:49-54.
- 23. Fridman WH. Regulation of cell activation and antigen presentation by Fc receptors (review). Curr Opin Immunol 1993;5:355-360.
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- 25. Blasczy KR, Westhoff U, Grosse-Wilde H. Soluble CD4, CD8 and HLA molecules in commercial immunoglobulin preparations. Lancet 1993;341:789-790.
- 26. Basta M, Dalakas MC. High-dose intravenous immunoglobulin exerts its beneficial effect in patients with dermatomyositis by blocking endomysial deposition of activated complement fragments. J Clin Invest 1994;94:1729-1735.
- 27. Dwyer JM. Manipulating the immune system with immune globulin. N Engl J Med 1992;326:107-116.
