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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.

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Objectives To compare Etanercept 50 mg subcutaneously (SC) weekly versus every other week in patients with rheumatoid arthritis (RA) who are in sustained remission. Methods Patients with established RA and in remission as determined by the Disease Activity Score (DAS) remission criteria (DAS28 (CRP)  2.6) for at least six months were randomized 1:1 to continued weekly or tapered every other week treatment of Etanercept 50 mg SC. Treatment modifications were predefined if remission status was lost and NSAID treatment was not sufficient to restore disease control in two weeks. Restarting the weekly dose was the first step in the tapering arm. The primary outcome was the proportion of patients in remission at 6 months compared between the two arms. Secondary endpoints were the proportions of remission at 12 months, sustained remission for 12 months, the proportion and time to regain remission after a flare with a re-escalated weekly dose and possible baseline predictors for remission. Results Data from 37 patients in the weekly and 36 in the every other week arm were analysed. At 6 months, 83.8% of the weekly and 83.3% of the every other week arm were in remission (p=0.959). Eleven patients of the every other week group had to be re-escalated to a weekly dose and regained their remission state mostly fast. Younger age was a possible predictor for remission at month 6 (p=0.023). No statistically significant differences were shown in other secondary endpoints. Conclusion For patients with established RA, the TapERA trial showed that tapering Etanercept 50 mg SC to an every other week regimen is possible while keeping remission. If losing disease control, patients re-escalated to a weekly dose regain remission fast.