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01-06-2016 | Orphan Diseases (B Manger, Section Editor)

SAPHO Syndrome: Current Developments and Approaches to Clinical Treatment

Authors: Davide Firinu, Vanessa Garcia-Larsen, Paolo Emilio Manconi, Stefano R. Del Giacco

Published in: Current Rheumatology Reports | Issue 6/2016

Abstract

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare autoimmune disease which, due to its clinical presentation and symptoms, is often misdiagnosed and unrecognized. Its main features are prominent inflammatory cutaneous and articular manifestations. Treatments with immunosuppressive drugs have been used for the management of SAPHO with variable results. To date, the use of anti-TNF-α agents has proved to be an effective alternative to conventional treatment for unresponsive or refractory SAPHO cases. TNF-α is a pro-inflammatory cytokine and pivotal regulator of other cytokines, including IL-1 β, IL-6, and IL-8, involved in inflammation, acute-phase response induction, and chemotaxis. IL-1 inhibition strategies with anakinra have shown efficacy as first and second lines of treatment. In this review, we will describe the main characteristics of biological drugs currently used for SAPHO syndrome. We also describe some of the promising therapeutic effects of ustekinumab, an antibody against the p40 subunit of IL-12 and IL-23, after failure of multiple drugs including anti-TNF-α and anakinra. We discuss the use and impact of the new anti-IL-1 antagonists involved in the IL-17 blockade, in particular for the most difficult-to-treat SAPHO cases.

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Title: SAPHO Syndrome: Current Developments and Approaches to Clinical Treatment
Authors: Davide Firinu
Vanessa Garcia-Larsen
Paolo Emilio Manconi
Stefano R. Del Giacco
Publication date: 01-06-2016
Publisher: Springer US
Published in: Current Rheumatology Reports / Issue 6/2016
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI: https://doi.org/10.1007/s11926-016-0583-y

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