Rare forms of inflammatory myopathies - part I, generalized forms

The idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, and inclusion body myositis. In this review, we aimed to cover the less common forms of generalized myositis.

We identified rare forms of widespread myositis on the basis of list provided by the homepage of the Neuromuscular disease center of Washington University, USA and on the basis of the authors' knowledge. We searched PubMed® and EMBASE® for relevant articles on these forms with the aim of providing as much as possible information on their clinical manifestations as well as guidance on their work-up and treatment.

There is substantial heterogeneity among the various rare forms of generalized myositis in terms of their frequency and characterization. Some forms are reasonably well defined, while others may not represent truly well-defined diseases, but rather variants of other myopathies. The landscape of rare forms appears to have evolved over time, with some forms now being better characterized, while others, such as SARS-Cov-2- and immune checkpoint inhibitor-related myositis have come to the fore only in recent years. Knowledge about rare forms of myositis can aid in their recognition and treatment.