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Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO): An Interesting Clinical Syndrome
Ali Hussain1, Mohsin Gondal2, Nizar Abdallah3
1Acute Medicine, Pinderfields General Hospital, Wakefield, GBR.
Abstract:
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare disorder that classically involves the musculoskeletal system, i.e. bone, joint and skin. The exact pathogenesis of this syndrome is unknown. However, autoimmunity, infections, immune malfunction and genetic factors are attributed to its pathophysiology. Bone and joint involvements are the hallmark of SAPHO syndrome and not necessarily require cutaneous involvement at the time of diagnosis. X-ray of the affected joints could show osteitis with sclerosis. Anterior chest wall involvement particularly ''bullhead appearance'' seen on bone scan is a striking feature of the syndrome. Erythrocyte sedimentation rate (ESR) is usually elevated amongst the majority of patients. Diagnosis of SAPHO is always challenging and often delayed because of a multitude of symptoms. The mainstay of treatment is control of pain and inflammation with both non-steroidal anti-inflammatory drugs (NSAIDs) and rescue courses of systemic steroids. If failed to control symptoms with first-line agents and in those with severe disease, disease-modifying anti-inflammatory drugs (DMARDs) may be needed eventually. Despite a chronic inflammatory condition, it remains stable in the majority of cases. Here in this case report, we reiterate the importance of early recognition, timely diagnosis and prompt treatment initiation.
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