Background/Purpose: The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare autoimmune disease characterized by inflammatory osteoarticular and cutaneous manifestations. Despite improvements in diagnostic (e.g. MRI, scintigraphy) and therapeutic options (e.g. NSAR, bisphosphonates and TNF inhibitors) SAPHO syndrome is still diagnosed late and often treatment is challenging. Since data from larger studies are missing we assessed patient characteristics and treatment in our cohort.

Methods:  69 Patients with CRMO were analyzed in a cross-sectional study using medical records and questionnaires with regard to the patient characteristics (age, gender, disease duration, time to diagnosis), perception of satisfaction and disease burden (VAS), clinical manifestations (osteoarticular: osteitis, hyperostosis, spondylitis, and involvement of sternoclavicular and sacroiliac joints; dermatological: acne, palmoplantar pustulosis, psoriasis vulgaris), and treatment modalities (NSAIDs, opioids, steroids, bisphosphonates, antibiotics, biologicals currently or in the past).

Results:  The time from onset to diagnosis was 4.0 ± 5.5 years and the age at diagnosis was 46.8 ± 12.8 years. Generally, the patients’ overall satisfaction (on visual analogue scale from 0 to 100) with 22.5 ± 27.8 was rather low and overall disease burden with 43.7 ± 24.8 rather high indicating that treatment was suboptimal in a substantial portion of patients. Among other findings, we identified a subgroup of difficult-to-treat patients that were characterized by osteitis of the mandibles (n=9). They experienced stronger immunosuppressive therapies such as steroids (p=0.03) and TNFi (p<0.005) than patients without mandible involvement. Of note, in three patients of our cohort who failed treatment including TNFi, we observed a significant improvement after treatment with secukinumab in patient pain, skin manifestation as well as in objective measures of osteoarticular inflammation as demonstrated by decrease of CRP and reduction of activity by MRI and bone scintigraphy.

Conclusion: Treatment of SAPHO is challenging particular in subgroups of patients. Therapies targeting IL-17 might constitute new efficacious treatment options.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/new-treatment-option-for-sapho/