TY - JOUR
T1 - SAPHO Syndrome
T2 - Current Developments and Approaches to Clinical Treatment
AU - Firinu, Davide
AU - Garcia-Larsen, Vanessa
AU - Manconi, Paolo Emilio
AU - Del Giacco, Stefano R.
N1 - Publisher Copyright:
© 2016, Springer Science+Business Media New York.
PY - 2016/6/1
Y1 - 2016/6/1
N2 - 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.
AB - 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.
KW - Anakinra
KW - Autoinflammatory
KW - Biologicals
KW - CRMO
KW - Canakinumab
KW - Cytokine
KW - Inflammation
KW - Interleukin-1β
KW - Osteitis
KW - T17
KW - TNF
KW - Ustekinumab
UR - http://www.scopus.com/inward/record.url?scp=84964289857&partnerID=8YFLogxK
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U2 - 10.1007/s11926-016-0583-y
DO - 10.1007/s11926-016-0583-y
M3 - Review article
C2 - 27108452
AN - SCOPUS:84964289857
VL - 18
JO - Current Rheumatology Reports
JF - Current Rheumatology Reports
SN - 1523-3774
IS - 6
M1 - 35
ER -