The Synovitis, Acne, Pustulosis, Hyperostosis, And Osteitis (SAPHO) syndrome: A two-center study of 23 patients

Introduction: The Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) syndrome is a rare autoinflammatory disease. The clinical presentation is heterogeneous, hence the difficulty of making the diagnosis. Aim of the work: To assess clinical, laboratory parameters and radiological features, and to detail the therapeutic options of SAPHO syndrome. Patients and methods: Patients diagnosed with SAPHO syndrome were retrospectively studied from two rheumatology centres in Tunisia. The epidemiological, clinical, radiological, and therapeutic data were collected from patients’ medical records. Results: Twenty-three patients were enrolled. The mean age was 44.7 ± 16.9 years (14–76 years). Skin involvement was found in a third of patients. Osteoarticular manifestations involved anterior chest wall damage in 56% of cases and axial skeletal lesions in 93% of patients. Ten (43%) patients had peripheral joint involvement, and one reported bone pain in the forearm. HLA-B27 was positive in a third of the patients. All patients were treated with nonsteroidal anti-inflammatory drugs (NSAIDs) with variable clinical responses. Antibiotic therapy with doxycycline was prescribed in 3 cases, with no clinical improvement. Sulfasalazine was used in 4 cases after therapeutic failure of NSAIDs. Methotrexate was prescribed in 4 cases. Tumor necrosis factor (TNF) inhibitors (Etanercept) were provided for 2 with a good clinical response, and were planned for two others. Pamidronate was given to a patient with bone involvement with pain relief and clinical improvement. Conclusion: Patients with SAPHO were relatively young. Skin lesions were not common. The involvement of the anterior chest wall and the axial skeleton was predominant.