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滑膜炎、痤疮、脓疱病、骨肥厚、骨炎(SAPHO):一种有趣的临床综合征。

Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO): An Interesting Clinical Syndrome.

作者信息

Hussain Ali, Gondal Mohsin, Abdallah Nizar, Yousuf Hira, Iqbal Mubashar

机构信息

Acute Medicine, Pinderfields General Hospital, Wakefield, GBR.

Cardiology, The Mid Yorkshire Hospitals NHS Trust, Wakefield, GBR.

出版信息

Cureus. 2020 Sep 1;12(9):e10184. doi: 10.7759/cureus.10184.

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.

摘要

滑膜炎、痤疮、脓疱病、骨肥厚和骨炎(SAPHO)综合征是一种罕见的疾病,典型地累及肌肉骨骼系统,即骨骼、关节和皮肤。该综合征的确切发病机制尚不清楚。然而,自身免疫、感染、免疫功能紊乱和遗传因素被认为与其病理生理学有关。骨骼和关节受累是SAPHO综合征的标志,诊断时不一定需要皮肤受累。受累关节的X线检查可显示伴有硬化的骨炎。骨扫描显示的前胸壁受累,特别是“牛头外观”,是该综合征的一个显著特征。大多数患者的红细胞沉降率(ESR)通常会升高。SAPHO的诊断总是具有挑战性,而且由于症状众多,往往会延迟。治疗的主要方法是使用非甾体抗炎药(NSAIDs)控制疼痛和炎症,并使用全身性类固醇进行挽救性治疗。如果一线药物无法控制症状,对于重症患者,最终可能需要使用改善病情的抗风湿药(DMARDs)。尽管是一种慢性炎症性疾病,但在大多数情况下病情仍保持稳定。在本病例报告中,我们重申了早期识别、及时诊断和迅速开始治疗的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/538d/7465995/f61aef8f0a9e/cureus-0012-00000010184-i01.jpg

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