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; Yao syndrome; amyopathic dermatomyositis; dermatomyositis; periodic fever syndrome.
© 2024 Saika Sharmeen, Lisa Christopher-Stine, Joann N. Salvemini, Peter Gorevic, Richard Clark, Qingping Yao, published by De Gruyter on behalf of NCRC-DID.