Idiopathic inflammatory myopathies are the group of rare heterogeneous autoimmune diseases of unknown etiology, characterized by inflammatory lesions of the striated muscles and skin, as well as the development of specific organ pathology. The history of the study idiopathic inflammatory myopathies has more than 100 years. Initially, this group of diseases included only 2 main subtypes: dermatomysitis and polymyositis. The latter was the main subtype of myopathies, it corresponded to 2/3 of the clinical descriptions of this pathology available at that time. The discovery of myositis-specific and myositis-associated autoantibodies at the end of the 20th century made it possible to significantly expand the understanding of the pathogenesis of idiopathic inflammatory myopathies. Not only the autoimmune genesis of myopathies was proven, but also their immunological heterogeneity, as well as the existence of clinical and immunological subtypes.Today, with no serological markers, no skin lesions, and no unique histological presentation, polymyositis is a difficult-to-diagnose disease that can often be misdiagnosed in patients with another pathology or subtype of idiopathic inflammatory myopathies. According to most experts, polymyositis is currently a diagnosis of exclusion and is the rarest subtype of idiopathic inflammatory myopathies, accounting to no more than 5 % of all cases in this group.
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