Department of Community and Global Health, Graduate School of Medicine, The University of Tokyo, Japan.
Health Research and Social Development Forum (HERD), P.O. Box 24133, Thapathali, Kathmandu, Nepal.
J Dermatol Sci. 2018 Apr;90(1):21-26. doi: 10.1016/j.jdermsci.2017.12.012. Epub 2017 Dec 28.
Primary hypertrophic osteoarthropathy (PHO), also known as pachydermoperiostosis is a rare genetic disease which predominantly affects skin, bone and soft connective tissue. It is characterized by the triad of pachydermia, digital clubbing and periostosis of long bones. Arthralgia or arthritis is also present in most of the cases. Genetic studies have identified the impaired PGE2 metabolism as a culprit for hypertrophic osteoarthropathy in PHO cases. We conducted a systematic review to examine the effectiveness of Non-Steroidal Anti-Inflammatory Drugs (NSAIDs), a PGE2 synthesis blocker to reduce the symptoms among PHO patients.
We searched the evidence in five databases; Cochrane Library, CINAHL, EMBASE, MEDLINE, and PubMed. We reported the evidence using narrative synthesis.
Out of 238 identified studies, we selected 26 for the synthesis. All were case reports which included a total of 54 patients. Among them, 39 patients were treated with at least one type of NSAIDs. Around 70% of the patients treated with NSAIDs had clinical improvement for their symptoms, mostly arthritis or arthralgia symptoms.
NSAIDs were effective in improving arthralgia or arthritis symptoms in majority of the PHO patients. Therefore, we recommend the use of NSAIDs in PHO patients to treat arthralgia or arthritis.
原发性肥大性骨关节病（PHO），又称厚皮性骨膜病，是一种罕见的遗传性疾病，主要影响皮肤、骨骼和软结缔组织。其特征为三联征，即皮肤增厚、杵状指（趾）和长骨骨膜增生。大多数病例还存在关节痛或关节炎。遗传研究表明，前列腺素 E2（PGE2）代谢受损是 PHO 患者发生肥大性骨关节病的罪魁祸首。我们进行了一项系统评价，以检查非甾体抗炎药（NSAIDs），即 PGE2 合成抑制剂，对减轻 PHO 患者症状的有效性。
我们在五个数据库中搜索证据，即 Cochrane Library、CINAHL、EMBASE、MEDLINE 和 PubMed。我们使用叙述性综合报告证据。
在 238 项已确定的研究中，我们选择了 26 项进行综合分析。这些研究均为病例报告，共纳入了 54 名患者。其中，39 名患者接受了至少一种 NSAIDs 的治疗。接受 NSAIDs 治疗的患者中，约 70%的患者症状得到了临床改善，主要是关节炎或关节痛症状。
NSAIDs 对大多数 PHO 患者的关节痛或关节炎症状有效。因此，我们建议在 PHO 患者中使用 NSAIDs 来治疗关节痛或关节炎。
