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Inmunoglobulina intravenosa en el síndrome de activación macrofágica asociado a lupus eritematoso sistémico / Intravenous immunoglobulin in macrophage activation syndrome associated with systemic lupus erythematosus
Resumen
Resumen
Introducción: La linfohistiocitosis hemofagocítica es una enfermedad de evolución potencialmente fatal, caracterizada por la activación de macrófagos y de histiocitos con hemofagocitosis en la médula ósea y en otros sistemas reticuloendoteliales, desencadenada por un defecto en los linfocitos T al estimular la producción de interleucina 1-beta, interleucina 6, interferón gamma y factor de necrosis tumoral alfa, que promueven la activación de los macrófagos. Esta afección cursa con fiebre, citopenias, esplenomegalia, hemofagocitosis en la médula ósea, hipertrigliceridemia e hipofibrinogenemia, en el contexto de una enfermedad infecciosa, neoplásica o autoinmunitaria. El objetivo de este artículo es describir la utilidad de la inmunoglobulina intravenosa (IgIV) en pacientes que no pueden recibir tratamiento inmunosupresor.
Casos clínicos: Se presentan dos casos de lupus eritematoso sistémico (LES) con síndrome de activación macrofágica (SAM), uno asociado a vasculitis del sistema nervioso central y otro a neutropenia febril, ambos con infección bacteriana agregada.
Conclusiones: El diagnóstico de SAM en pacientes con actividad lúpica se debe sospechar en caso de fiebre, citopenias, visceromegalias, hipertrigliceridemia e hipofibrinogenemia. Un diagnóstico y un tratamiento oportunos son importantes para disminuir de manera considerable la mortalidad. Se propone que el tratamiento de primera línea en los pacientes con LES asociado a SAM y sepsis sea la IgIV, y como segunda línea un inmunosupresor con un esteroide intravenoso.
Abstract
Background: The hemophagocytic lymphohistiocytosis is a disease with a potential fatal evolution, caused by the activation of macrophages and histiocytes with hemophagocytosis in bone marrow and other reticuloendothelial systems, triggered by a defect in the T lymphocyte when stimulating the production of interleukin 1-beta, interleukin 6, interferon-gamma and tumor necrosis factor-alpha that promote macrophage activation. This condition presents with fever, cytopenias, splenomegaly, hemophagocytosis in bone marrow, hypertriglyceridemia and hypofibrinogenemia, in the context of an infectious, neoplastic or autoimmune disease. The objective of this article is to describe the utility of intravenous immunoglobulin (IVIg) in patients unable to receive immunosuppressive treatment.
Case reports: We present two case reports of systemic lupus erythematosus (SLE) with macrophage activation syndrome (MAS), one of them associated with central nervous system vasculitis and the other one with febrile neutropenia, both with bacterial infection added.
Conclusions: The diagnosis of MAS should be suspected in all patients with lupus activity, fever, cytopenias, visceromegalies, hypertriglyceridemia and hypofibrinogenemia. Diagnosis and treatment are important to significantly reduce mortality. It is proposed that the first line treatment in patients that present SLE associated with MAS and sepsis should be IgIV and as a second line immunosuppressants with intravenous steroids.
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Referencias
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