Amyopathic Dermatomyositis May Be on the Spectrum of Autoinflammatory Disease: A Clinical Review

Authors

Saika Sharmeen
Lisa Christopher-Stine
Joann N Salvemini
Peter Gorevic
Richard Clark
Qingping Yao

Publication Date

3-1-2024

Journal

Rheumatology and Immunology Research

Abstract

Systemic autoinflammatory diseases (SAIDs) are distinct from autoimmune diseases. The former primarily results from abnormal innate immune response and genetic testing is crucial for disease diagnosis. Similar cutaneous involvement is a main feature for both SAID and dermatomyositis (DM), so they can be confused with each other. A literature search of PubMed and MEDLINE was conducted for relevant articles. The similarities and differences between these two types of diseases were analyzed. We found phenotypic similarities between these two types of disorders. Accumulating data supports a major role of the innate immune system and a similar cytokine profile. Molecular testing using an autoinflammatory disease gene panel may help identify SAID patients from the DM population and may offer therapeutic benefit using interleukin-1 (IL-1) inhibitors. A subset of DM, notably amyopathic dermatomyositis in the absence of autoantibodies may be on the spectrum of autoinflammatory disease.

Keywords

Autoinflammatory disease, dermatomyositis, amyopathic dermatomyositis, periodic fever syndrome, Yao syndrome

Comments

PMID: 38571935