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Autoimmune diseases result from an immune response against structures of the organism itself. Their etiology is unknown, although they have been associated with genetic and environmental factors and the participation of mast cells has recently been proposed. The objective of this review was to provide an update on the role of mast cells in these diseases.According to the literature, mast cells not only intervene in immune processes as a defense mechanism but are also involved in the development of disease. Thus, mast cells may participate in important ways in autoimmune diseases through the secretion of cytokines or release of enzymes from their cytoplasmic granules or through the activity of proinflammatory mediators released by these cells. Further research is warranted to elucidate the role of mast cells in autoimmune diseases.