[New developments in immunomodulation of idiopathic inflammatory myopathies]

Idiopathic inflammatory myopathies (IIM) can substantially differ with respect to manifestations and severity, which influences both the diagnostics and therapeutic management. The treatment comprises pharmacological and nonpharmacological approaches. Despite advances in clinical care, patients frequently experience refractory and severe disease courses, making continuous development and improvement of therapeutic management essential.

This article summarizes the current state and new developments in immunomodulatory therapy for IIM.

A narrative literature review was conducted focusing on current guidelines and recommendations as well as recent studies and developments in the pharmacological treatment of IIM.

The article is structured around three main topics: (1) an overview of guideline-based pharmacological treatment, (2) treatment for specific IIM subtypes, such as scleromyositis or inclusion body myositis and (3) new, potentially promising treatment approaches, including strategies targeting B cells, plasma cells and T cells, antibody recycling or interferon-directed treatment options.

Immunomodulatory therapy should always be embedded in a multimodal treatment concept and consistently complemented by nonpharmacological measures. Given the potentially high morbidity and mortality, rapid initiation of treatment and in some cases timely combination treatment, is required depending on the IIM subtype and disease course. Certain forms of IIM call for specific therapeutic strategies, for example steroid-sparing regimens to reduce the risk of renal crises in scleromyositis. Several new targeted approaches (including B cell, T cell and interferon-directed strategies) are currently undergoing clinical evaluation and offer hope for patients with treatment-refractory disease.