Idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIM) consist of rare heterogeneous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis. Despite many similarities, the IIM are fairly heterogeneous from the histopathologic and pathogenetic standpoints, and also show some clinical and treatment-response differences. The field has witnessed significant advances in our understanding of the pathophysiology and treatment of these rare disorders. This review focuses on dermatomyositis, polymyositis, and necrotizing myopathy, and examines current and promising therapies.

Dimachkie MM ，Barohn RJ ，Amato AA 《-》

[Idiopathic Inflammatory Myopathies].

Myositis is a rare and an extremely heterogeneous autoimmune disease, that causes muscle weakness. Currently, "idiopathic inflammatory myopathies (IIM)" is the preferred umbrella-term used to describe the disease complexity within individuals. IIM include dermatomyositis, polymyositis, inclusion body myositis, autoimmune necrotizing myopathy, overlap myositis and antisynthetase syndrome. Research activity concerning myositis was very intense over the past ten years and led to new diagnostic approach as well as to novel therapeutic strategies. Correct classification is the key for successful management. One single treatment regime for every possible organ involvement in all different forms of IIM is still not existing.

Tomaras S ，Kekow J ，Feist E 《-》

Idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIM) encompass a heterogeneous group of rare disorders that present with acute, subacute, or chronic muscle weakness. Besides overlapping clinical manifestations, polymyositis, dermatomyositis and autoimmune necrotizing myopathy may be associated with cancer or collagen vascular disease, and respond generally well to immunosuppressive therapy. However, these 3 IIM are divergent from the histopathological and pathogenetic standpoints. On the other hand, inclusion body myositis (IBM), the most common IIM in the elderly, is clinically, histopathologically and pathogenetically distinct. IBM is also refractory to all currently available therapies. In this manuscript, we depict advances in our knowledge of the IIM, with emphasis on clinical presentation, associated conditions, laboratory features, electrophysiology, muscle histopathology, pathogenesis, and therapy.

Dimachkie MM 《-》

Inflammatory muscle disease - An update.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of inflammatory myopathies whose common feature is immune-mediated muscle injury. There are distinct subgroups including dermatomyositis (DM), polymyositis (PM), inclusion body myositis, and immune-mediated necrotizing myopathy. Antisynthetase syndrome is also emerging as a distinct subgroup with its unique muscle histopathological characteristic of perifascicular necrosis. While the newly updated EULAR/ACR Classification Criteria for IIM have brought advancements in diagnosis and the exclusion of mimickers, the use of only one autoantibody in the derivation of the schema limits its use. Similarly, while the advent of multiple novel therapeutics in the treatment of myositis has been exciting, it has also highlighted the scarcity of validated outcome measures. The purpose of our review is to highlight the updated classification criteria of myositis, newly reported clinical phenotypes associated with myositis autoantibodies, the measurement of outcomes, and emerging treatments in the field.

Baig S ，Paik JJ 《-》

[New insights of myositis-specific and -associated autoantibodies in juvenile and adult type myositis].

Váncsa A ，Dankó K 《ORVOSI HETILAP》