SAPHO syndrome
ICD-10 M86.3 · ICD-11 4A61

Treatment of SAPHO Syndrome When TNF Inhibitor Therapy Has Not Worked

In patients with SAPHO syndrome whose disease remains active and refractory after TNF inhibitor treatment, a distinct next-line protocol addresses persistent skin and nail manifestations alongside elevated systemic inflammatory markers.

Prior TNF inhibitor therapy A course of adalimumab, etanercept, or infliximab — used for otherwise treatment-refractory SAPHO syndrome — did not achieve the required clinical response: meaningful reduction of pain, lowering of Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), and resolution of bone inflammation on imaging. This failure of response is the trigger for escalation to the next protocol.
  • Improvement of nail lesions, measured by Nail Psoriasis Severity Index (NAPSI)
  • Improvement of palmoplantar pustulosis, measured by Palmoplantar Pustulosis Area and Severity Index (PPASI)
  • Reduction of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)
The next-line protocol for TNF inhibitor–refractory SAPHO syndrome involves a kinase inhibitor class of therapy, shown to be of particular relevance for refractory skin and nail involvement. The complete regimen, selection criteria, and monitoring guidance are available in the full structured protocol.

References

DOI: 10.1136/rmdopen-2023-003688

One study investigated effects of the JAK inhibitor tofacitinib on dermatological manifestations in 13 Asian female SAPHO patients (12 weeks of 5 mg tofacitinib, twice daily).

Results were positive with significant benefit for nail lesions, palmoplantar pustulosis and associated quality of life.

Bisphosphonates may achieve rapid remission of bone inflammation and associated pain, cDMARDs and TNFi are effective for bone and (to some extent) skin involvement, while JAKi may be an option especially in otherwise treatment refractory skin and nail involvement.

While a reduction of systemic inflammatory parameters was observed (CRP and ESR), no information was provided on bone involvement.

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