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Introduction The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a rare chronic rheumatic disease which is characterized by chronic inflammatory osteoarticular manifestations in combination with cutaneous lesions. It is a highly heterogeneous disease; the symptoms are variable and they do not always occur synchronously. Due to this, the diagnosis can be challenging and delayed. The first purpose of this review is to summarize the current knowledge on clinical and radiological manifestations and the different treatment options for SAPHO syndrome. The second purpose is to create and enhance awareness for this rare and underdiagnosed condition. Methods Using three medical databases (Medline, Embase and Cochrane) a literature search on the SAPHO syndrome was performed and a review on current knowledge was made. Results The two main clinical features of the SAPHO syndrome consist of osteoarticular and cutaneous manifestations. The most common osteoarticular affected sites in adults are the anterior chest wall, the spine and the sacroiliac joints. The typical skin lesions found in SAPHO patients are palmoplantar pustulosis (PPP) and severe acne. Infectious, immunological and genetic components have been proposed to contribute to the development of the disease, but the exact pathophysiology is still unclear. Imaging plays a key role in the diagnosis of SAPHO syndrome and different imaging modalities can be used. The clinical course is marked by recurrent episodes of relapse and remission. Despite the rather good prognosis of the SAPHO syndrome, this disease and especially the associated pain, has a great influence on the quality of life of these patients. Conclusion Due to the heterogeneity, SAPHO syndrome is frequently underdiagnosed and misdiagnosed, causing delay in diagnosis and treatment. In order to facilitate an early diagnosis a multidisciplinary (rheumatologist, dermatologist, radiologist) approach is required. The diagnosis is straightforward when there is involvement of the axial skeleton (anterior chest wall, spine) and when the osteoarticular manifestations are associated with typical skin lesions, but if the presentation is atypical the diagnosis may be more challenging.