Treatment Options and Outcomes in Patients with Idiopathic Inflammatory Myopathies

Idiopathic inflammatory myopathies (IIM) are a very heterogeneous group of conditions, which can now be increasingly differentiated. This paves the way for targeted treatment. Therapies aim to change pathogenic mechanisms and are focused on reducing disease activity, encouraging muscle development, preventing organ damage, and improving quality of life. The following review summarises existing data on the treatments currently used and provides an overview of future alternatives. Glucocorticoids continue to be an indispensable component of the treatment of polymyositis (PM) and dermatomyositis (DM). A combination of drugs including conventional DMARDs has long been established. The ProDERM study provides a solid basis for the use of immunoglobulins in the clinical setting. In severe forms of the disease, rituximab is increasingly replacing cyclophosphamide. Promising early results obtained with abatacept, janus kinase- inhibitors, apremilast, sifalimumab and lenabasum must be corroborated by further studies. The treatment of extramuscular involvement is a challenging issue. The future value of nintedanib in the treatment of interstitial lung disease associated with myositis (IIM-ILD) is of significant interest. The management of inclusion body myositis (IBM) continues to be a major challenge. A number of studies conducted thus far have yielded no convincing evidence of any improvement in the prognosis of the disease. When a patient shows signs of treatment-refractory disease, it is high time for a clinician to consider cancer-associated myositis. In some cases, a hereditary myopathy may be hidden behind myositis triggered by infection or overexertion. Complications in the course of disease, such as dysphagia, infection or myocardial involvement, are not rare at all. Multimorbidity is quite common. Interdisciplinary cooperation is an absolute prerequisite for supporting and accompanying a patient successfully. A competent interdisciplinary team must include an experienced physiotherapist, an occupational therapist and a psychotherapist.