La Dermatomiositis

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.

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

Referencias Bibliográficas

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• 7. Boguniewicz M, Szelc KC, Ballow M, Leung DYM. Intravenous immune globulin in autoimmune and inflammatory diseases. In: Rich RR, Fleisher TA, Schwartz BD, Shearer WT, Strober W, eds. Clinical Immunology. Principles and Practice. St. Louis: Mosby, vol.II; 1996. p. 1891-1903.
• 8. Kazatchkine MD. Mechanism of action of intravenous immunoglobulin in immune-mediated diseases. In: Kazatchkine MD, Morell A, eds. Intravenous immunoglobulin research and therapy. New York: The Parthenon Publishing Group, 1996. p.29-41.
• 9. Ruiz de Souza V, Carreno MP, Kaveri SV, et al. Selective induction of interleukin-1 receptor antagonist and interleukin-8 in human monocytes by normal polyspecific IgG (intravenous immunoglobulin). Eur J Immunol 1995;25:1267-1273.
• 10. Abe Y, Horiuchi A, Miyake M, Kimura S. Anti-cytokine nature of human immunoglobulin: one possible mechanism of the clinical effect of intravenous therapy. Immunol Rev 1994;139:5-19.

Fuentes Bibliográficas

• 11. Anderson UG, Bjork L, Skansen-Saphir U, Andersson JP. Down-regulation of cytokine production and IL-2 receptor expression by pooled human IgG. Immunology 1993;79:211-216.
• 12. Takei S, Arora Y, Walker SM. Intravenous immunoglobulin contains specific antibodies inhibitory to activation of T cells by staphylococcal toxin superantigens. J Clin Invest 1993;91:602-607.
• 13. Vassilev T, Gelin C, Kaveri SV, Zilber MT, Boumsell L, Kazatchkine MD. Antibodies to the CD5 molecule in normal human immunoglobulins for therapeutic use (intravenous immunoglobulins, IVIg). Clin Exp Immunol 1993;92:369-372.
• 14. Hurez V, Kaveri SV, Mouhoub A, et al. Anti CD4 activity of normal human immunoglobulins G for therapeutic use (intravenous immunoglobulin, IVIg). Therap Immunol 1994;1:269-278.
• 15. Kaveri S, Vassilev T, Hurez V, et al. Antibodies to a conserved region of HLA class I molecules, capable of modulating CD8-T cell mediated function, are present in pooled normal immunoglobulin for therapeutic use (IVIg). J Clin Invest 1996;97:865-869.
• 16. Imbach P, d’Apuzzo V, Baumgartne R. High dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood. Lancet 1981;1:1228-1230.
• 17. Leung DYM, Burns JC, Newburger JW, Geha RS. Reversal of lymphocyte activation in vitro in the Kawasaki syndrome by intravenous gammaglobulin. J Clin Invest 1987;79:468-472.
• 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.
• 24. Ashman R, Peckham D, Stunz L. Fc receptor off-signal in the B cell involves apoptosis. J Immunol 1996;157:5-11.
• 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.