Thrombotic microangiopathy is a group of syndromes characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute organ dysfunction secondary to ischemia. It includes a group of diseases such as thrombotic thrombocytopenic purpura. They can be primary or secondary to multiple pathologies, including autoimmune diseases such as systemic lupus erythematosus. These associations are important from the clinical point of view, as they have more severe presentations, high relapse rates, and in some cases poor response to conventional treatment.