Biological Treatments for SAPHO Syndrome: An Update
Davide Firinu, Giuseppe Murgia, Maria Maddalena Lorrai, Maria Pina Barca, Maria Monica Peralta, Paolo Emilio Manconi and Stefano R. del Giacco
Affiliation: Department of Medical Sciences “M. Aresu”, Unit of Internal Medicine, Allergy and Clinical Immunology, University of Cagliari, Azienda Ospedaliero Universitaria, SS 554-Bivio Sestu, I-09042 Monserrato (CA), Italy.
Keywords: Anakinra, autoinflammatory, biologicals, canakinumab, CRMO, cytokine, inflammation, interleukin-1β, osteitis,
TH17, TNF, ustekinumab.
Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) syndrome is a rare and often unrecognized
disease with prominent inflammatory cutaneous and articular manifestations. Since the identification of the syndrome
many immunosuppressive drugs have been used for the management of SAPHO, with variable results. The use of anti-
TNF-α agents as a therapeutic option for SAPHO cases unresponsive or refractory to conventional drugs, demonstrated
their efficacy for bone, skin and joints manifestations. 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 proven their efficacy as first and second line treatment. We
herein review the literature concerning the use of biological drugs in patients with SAPHO syndrome. In addition, we
describe for the first time the use 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. This anti-IL12/IL23 agent could be a promising therapeutic option,
also considering the opportunity to interfere with the IL23/TH17 pathway, which we recently found disturbed.
Furthermore, a rationale emerges for the use of the new anti-IL-1 antagonists or the IL-17 blockade, in particular for the
most difficult-to-treat SAPHO cases.
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