SAPHO Syndrome: Current Developments and Approaches to Clinical Treatment.
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.
Internal Medicine, Allergy and Clinical Immunology, Department of Medical Sciences "M. Aresu", University of Cagliari, Asse Didattico "E1" - Cittadella Universitaria, 09042, Monserrato, Cagliari, Italy.
Davide
D
Firinu
Respiratory Epidemiology, Occupational Medicine and Public Health Group, National Heart and Lung Institute, Imperial College London, Manresa Road, SW3 6LR, London, UK.
Vanessa
V
Garcia-Larsen
Internal Medicine, Allergy and Clinical Immunology, Department of Medical Sciences "M. Aresu", University of Cagliari, Asse Didattico "E1" - Cittadella Universitaria, 09042, Monserrato, Cagliari, Italy.
Paolo Emilio
PE
Manconi
Internal Medicine, Allergy and Clinical Immunology, Department of Medical Sciences "M. Aresu", University of Cagliari, Asse Didattico "E1" - Cittadella Universitaria, 09042, Monserrato, Cagliari, Italy. stedg@medicina.unica.it.
Stefano R
SR
Del Giacco
D020083:Acquired Hyperostosis Syndrome / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D000068879:Adalimumab / Q000627:therapeutic use; D001688:Biological Products / Q000627:therapeutic use; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D007375:Interleukin-1 / Q000037:antagonists & inhibitors; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors; D000069549:Ustekinumab / Q000627:therapeutic use
D001688:Biological Products; D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1; D014409:Tumor Necrosis Factor-alpha; D000069549:Ustekinumab; D000068879:Adalimumab
Anakinra; Autoinflammatory; Biologicals; CRMO; Canakinumab; Cytokine; Inflammation; Interleukin-1β; Osteitis; TH17; TNF; Ustekinumab