Biologics in SAPHO syndrome: A systematic review

Semin Arthritis Rheum. 2019 Feb;48(4):618-625. doi: 10.1016/j.semarthrit.2018.04.003. Epub 2018 Apr 17.


Background: The SAPHO syndrome is a relatively rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Biologics have been used in cases refractory to conventional treatment.

Methods: We present herein a patient with refractory to treatment SAPHO syndrome who exhibited a dramatic and fast response to IL-17 blockade. Additionally, we performed a systematic review of all cases of patients with SAPHO syndrome treated with biologics to date.

Results: We identified 66 cases treated with biologics (45 with TNF blockers, 7 with IL-1 blockers, 13 with biologics targeting the IL-23/IL-17 axis, and 1 with tocilizumab). Data support a positive effect of anti-TNF treatment in SAPHO with a response rate in bone and joint manifestations of 93.3%. Skin disease also improved in 21/29 cases (72.4%). Data related to IL-1 inhibition in SAPHO are encouraging with most patients exhibiting a significant response in musculoskeletal manifestations (6/7, 85.7%). However, IL-1 inhibition is not effective in skin manifestations. Ustekinumab seems to have some efficacy with 2/4 patients responding in skin and 3/5 in bone/joint manifestations. Data related to IL-17 blockade indicate efficacy in skin disease with 4/7 patients responding (57.1%). Joint/bone manifestations improved in 3/8 patients (37.5%).

Conclusions: In SAPHO patients not responding to conventional treatment, TNF blockers appear to be the first choice. In patients failing TNF blockers, IL-1 inhibitors and biologics targeting the IL-17/IL-23 axis could be used.

Keywords: Abatacept; Adalimumab; Anakinra; Anti-TNF; Biologics; Canakinumab; Certolizumab; Etanercept; Golimumab; IL-1; IL-17; IL-23; IL-6; Infliximab; Rituximab; SAPHO; Secukinumab; Treatment; Ustekinumab; tocilizumab.

Publication types

  • Systematic Review

MeSH terms

  • Acquired Hyperostosis Syndrome / drug therapy*
  • Biological Products / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Treatment Outcome


  • Biological Products
  • Immunosuppressive Agents