Inflammatory muscle involvement in systemic vasculitis: A systematic review

Autoimmun Rev. 2022 Mar;21(3):103029. doi: 10.1016/j.autrev.2021.103029. Epub 2021 Dec 29.


Vasculitis are severe systemic autoimmune diseases which may involve different organs and systems. Conversely, muscles do not represent an organ commonly involved by systemic vasculitis and myositis is not include among any classification or diagnostic criterion of vasculitis. In this regard, we aimed to review the literature in order to report all the available evidence concerning the inflammatory involvement of muscle in patients affected by systemic vasculitis. We collected a total of 108 papers, for a sum of 395 patients affected by muscle vasculitis. Most of them suffered from medium and small vessels vasculitis (mainly polyarteritis nodosa and ANCA-associated vasculitis) or from vasculitis secondary to rheumatoid arthritis. Conversely, muscle involvement in case of large vessel vasculitis occurred seldom, while only few papers reported such occurrence in Kawasaki or Behçet's disease. Histological findings may differ, but the most common ones displayed a necrotizing vasculitis of perimysium vessels, while granulomatous vasculitis was assessed only in case of ANCA-associated vasculitis patients. Creatine kinase were usually within normal range, seldom elevated, while imaging findings were generally undistinguishable from the ones found in idiopathic inflammatory myopathies: magnetic resonance imaging displays signal hyperintensity in T2 and STIR scans, while few data exist for positron emission tomography. The presentation of the disease may be fearsome and severe, sometimes life-threatening, but an overall good response to conventional immunosuppressants and/or glucocorticoids has been reported.

Keywords: Autoimmunity; Inflammation; Muscle; Myopathy; Myositis; Vasculitis.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / complications
  • Arteritis*
  • Behcet Syndrome* / diagnosis
  • Humans
  • Muscles / pathology
  • Polyarteritis Nodosa*