In most of the cases, corticosteroids represent the first line therapy of systemic autoimmune diseases. However, because of the multiple secondary effects occurring in patients treated with corticosteroids, and because their prevalence is positively correlated with the cumulative dose, daily dose and the age of the patient, a number of strategies have been proposed to reduce the corticosteroid dose including therapeutic association with immunosuppressants and/or immunomodulatory agents. Indications of immunosuppressants vary depending on clinical manifestations and pathogenesis of the disease concerned. Sometimes, immunosuppressants can be prescribed as a first line therapy alone or in association with corticosteroids; in other cases, they can be prescribed as a second line therapy in case of failure of corticosteroid therapy or in case of failure to taper the corticosteroid dose due to disease flare. The choice of the best immunosuppressant depends on the disease and is ideally made on a pathophysiology basis. In this review article, we will review therapeutic indications, modalities of administration, mechanisms of action and adverse events of the main immunosuppressants and/or immunomodulatory agents prescribed in systemic autoimmune diseases.
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