The Role of Myositis-Specific Autoantibodies in the Dermatomyositis Spectrum.

Harshita Marasandra Ramesh,Shravya Venugopal,Nikhil Chowdary Peddi,Sai Sreeya Gude,Sravya Vuppalapati,Sai Sravya Gude

doi:10.7759/cureus.22978

Abstract

Dermatomyositis (DM) is a systemic autoimmune disease that affects skeletal muscles, the skin, and the lungs. It is characterized by autoantibodies, tissue inflammation, parenchymal cell damage, death, and vasculopathy. In terms of epidemiology, DM affects both children and adults. The current pathophysiology of DM is described as an autoimmune attack on the afflicted organs driven by environmental variables such as UV exposure, medications, infections, and lifestyle choices in genetically predisposed people. DM is also a paraneoplastic condition, which means that cancer may arise before, along with, or following the development of the symptoms of DM.Myositis-specific autoantibodies are associated with phenotypical features and are used for sub-classification of dermatomyositis patients. Because the risk of interstitial lung disease (ILD), internal malignancy, destructive disease trajectory, and maybe a response to medication differs by DM myositis-specific antibody (MSA) group, a better knowledge of MSAs and the validation and standardization of tests employed for detection is crucial for improving diagnosis and treatment. The diagnostic sensitivity and specificity of tests for various MSAs are not ideal, just like with any other test. However, more antibody tests are anticipated to make their way into formal schemata for diagnosis and actionable risk assessment in DM due to worldwide standardization and more extensive research. In this review, we outline crucial aspects for interpreting clinical and pathologic relationships with MSA in DM and critical knowledge and practice gaps that will optimize the clinical benefit and utility of MSAs as diagnostic and prognostic markers.

Highlights

BackgroundIdiopathic inflammatory myopathies (IIMs) are a group of illnesses that affect the systemic skeletal muscles and are frequently associated with extramuscular complications such as interstitial lung disease (ILDs), arthritis, and cancer
The Bohan and Peter criteria [4,5], proposed in 1975, have been widely used to identify patients; they do not adequately describe the full spectrum of myositis and do not provide a definition for clinically amyopathic myositis, immune-mediated necrotizing myositis (IMNM), or inclusion body myositis, which is not seen in children [3]
Because the illness is a paraneoplastic syndrome, proper screening efforts in people with contemporaneous or metachronous malignancy will be possible if the skin signs are recognized

Summary

Introduction

Idiopathic inflammatory myopathies (IIMs) are a group of illnesses that affect the systemic skeletal muscles and are frequently associated with extramuscular complications such as interstitial lung disease (ILDs), arthritis, and cancer. Anti-Mi-2 autoantibodies have been seen in up to 21% of people with pDM [29] In contrast to this normal presentation of DM, patients may experience rapid progression of symptoms over a considerably shorter period of time, with the majority of patients reporting cutaneous signs followed by rapid muscle degeneration [30]. Anti-TIF proteins are overexpressed in numerous cancer forms, as well as the fact that an autoimmune response to TRIM28 is a common finding in patients with colorectal carcinoma (even without DM), backs up this theory [42] It is only found in 23-29% of juvenile DM patients [34] and is one of the most commonly found antibodies in juvenile DM, but it is not linked to malignancy, and patients under the age of 40 have no increased risk of malignancy even though they possess the antibody [43]. Using a universal "platform" for MSA detection and acquiring data from larger prospective and diverse patient cohorts will enhance our interpretation of the significance of serological data in DM [14]

Conclusions

Findings

Disclosures