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

IntroductionThe 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 workTo assess clinical, laboratory parameters and radiological features, and to detail the therapeutic options of SAPHO syndrome.Patients and methodsPatients 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.ResultsTwenty-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.ConclusionPatients with SAPHO were relatively young. Skin lesions were not common. The involvement of the anterior chest wall and the axial skeleton was predominant.