鲁昕, 杨波, 仉建国. 5例厚皮骨膜增生症合并滑膜炎的临床特征分析[J]. 罕见病研究, 2023, 2(4): 523-528. DOI: 10.12376/j.issn.2097-0501.2023.04.009
引用本文: 鲁昕, 杨波, 仉建国. 5例厚皮骨膜增生症合并滑膜炎的临床特征分析[J]. 罕见病研究, 2023, 2(4): 523-528. DOI: 10.12376/j.issn.2097-0501.2023.04.009
LU Xin, YANG Bo, ZHANG Jianguo. 5 Cases Analysis of Pachydermoperiostosis Complicated with Synovitis[J]. Journal of Rare Diseases, 2023, 2(4): 523-528. DOI: 10.12376/j.issn.2097-0501.2023.04.009
Citation: LU Xin, YANG Bo, ZHANG Jianguo. 5 Cases Analysis of Pachydermoperiostosis Complicated with Synovitis[J]. Journal of Rare Diseases, 2023, 2(4): 523-528. DOI: 10.12376/j.issn.2097-0501.2023.04.009

5例厚皮骨膜增生症合并滑膜炎的临床特征分析

5 Cases Analysis of Pachydermoperiostosis Complicated with Synovitis

  • 摘要:
      目的  通过分析厚皮骨膜增生症(PDP)合并滑膜炎患者的临床特点、治疗及预后,提高临床医师对PDP的认识。
      方法  对2010年1月至2015年6月在北京协和医院确诊为PDP合并滑膜炎的5例患者临床资料进行回顾性分析。
      结果  本研究的5例患者均为男性,平均年龄(24.2±3.8)岁。PDP均以关节肿痛、皮肤增厚为主要表现。本研究中4例患者接受了药物治疗,主要为非甾体类抗炎药(NSAIDs),联合微创关节镜下滑膜切除术治疗,治疗后关节症状明显改善。最后1例患者因滑膜炎症状较轻,仅应用药物保守治疗后明显缓解。
      结论  PDP合并滑膜炎的诊治仍具有挑战性,误诊率较高。根据患者症状程度,选择合适的保守或微创手术治疗,辅以NSAIDs口服,可获得较为满意的疗效。NSAIDs在某些患者的治疗中有望控制病情进展。

     

    Abstract:
      Objective  To analyze the clinical characteristics, treatments and prognosis of pachydermoperiostosis (PDP) with synovitis, and to improve clinicians′ understanding of PDP.
      Methods  The clinical data of 5 patients diagnosed with PDP in Peking Union Medical College Hospital from January 2010 to June 2015 were retrospectively analyzed.
      Results  Among the 5 patients in this study, all were male, with an average age of (24.2±3.8)years old. The main manifestations of PDP were joint swelling and pain and skin thickening. In this study, all 5 patients were treated with medication, including non-steroidal anti-inflammatory drugs, glucocorticoids, and immunosuppressants, and 4 patients were treated with arthroscopic minimally invasive synovectomy. Their symptoms gradually improved and controlled. The last patient′s symptoms of synovitis were mild and significantly relieved after conservative treatment.
      Conclusions  The diagnosis of PDP is still challenging. Symptoms can be alleviated, and the progression of the disease can be basically controlled after active drug therapy and surgical mitigation measures.

     

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