, Volume 78, Issue 12, pp 1187–1195 | Cite as

Adult-Onset Still’s Disease: Molecular Pathophysiology and Therapeutic Advances

  • Paolo SfrisoEmail author
  • Sara Bindoli
  • Paola Galozzi
Leading Article


Adult-onset Still’s disease (AOSD) is a rare inflammatory disorder of unknown etiology generally characterized by persistent high spiking fever, evanescent rash, and polyarthritis. The pathogenesis of AOSD is only partially known. The pivotal role of macrophage cell activation, which leads to T-helper 1 (Th1) cell cytokine activation, is now well-established in AOSD. Moreover, pro-inflammatory cytokines such as interleukin (IL)-1, -6, and -18 seem to play a key role in this disorder, giving rise to the development of new targeted therapies. For years, treatment of AOSD has been largely empirical, using nonsteroidal anti-inflammatory drugs, corticosteroids, and disease-modifying antirheumatic drugs. Patients with steroid- and methotrexate-refractory AOSD can now benefit from efficient and well-tolerated biologic agents such as IL-1, IL-6, and tumor necrosis factor-α antagonists.


Compliance with Ethical Standards


No sources of funding were used to conduct this study or prepare this manuscript.

Conflict of interest

Paolo Sfriso, Sara Bindoli, and Paola Galozzi have no conflicts of interest.


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© Springer Nature Switzerland AG 2018

Authors and Affiliations

  1. 1.Rheumatology Unit, Department of Medicine DIMEDUniversity of PadovaPaduaItaly

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