How I treat idiopathic patients with inflammatory myopathies in the clinical practice

•Idiopathic inflammatory myopathies (IIMs) are heterogeneous and rare diseases often requiring challenging management.•There is a compelling need for standardized treatment regimens for all disease manifestations of IIMs.•The mainstay of treatment is represented by glucocorticoids in most organ involvement.•Refractory cases are frequent and immunosuppressants are often needed to control disease manifestations.•Promising results have been obtained with intravenous immunoglobulins and rituximab, particularly in refractory or life-threatening manifestations.

Management of patients with idiopathic inflammatory myopathies (IIMs) is challenging given the systemic nature of the disease, which is often complicated by potentially life-threatening manifestations and the lack of standardized treatment regimens. Aim of this review is to provide the currently available evidence for immunotherapy in the treatment of various manifestations of IIM in order to help clinicians in the daily management of these patients.