Amyopathic dermatomyositis may be on the spectrum of autoinflammatory disease: A clinical review

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

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Pages (from-to)42-48
Number of pages7
JournalRheumatology and Immunology Research
Issue number1
StatePublished - 1 Mar 2024
Externally publishedYes


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


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