Vaidya Binit, Baral Rikesh, Baral Hritu, Nakarmi Shweta
Department of Rheumatology, National Center for Rheumatic Diseases(NCRD), Kathmandu 44600, Nepal.
Oxf Med Case Reports. 2019 Apr 16;2019(4):omy128. doi: 10.1093/omcr/omy128. eCollection 2019 Apr.
Pachydermoperiostosis is a rare genetic disorder characterized by skin thickening, digital clubbing and periostitis. The pathogenesis is incompletely understood and there are no proven treatments for its manifestations. Although arthritis has been reported in 20-40% cases, most are non-inflammatory in nature and usually treated symptomatically with steroids or NSAIDs. We report two cases of pachydermoperiostosis with inflammatory variant of arthritis and raised inflammatory markers who were treated with tapering dose of prednisolone for 6 weeks and maintained on long-term low dose methotrexate like rheumatoid arthritis and followed for 2 years. In both cases, methotrexate was well tolerated and helped in maintaining symptomatic improvement and slowed the disease progression with significant steroid and NSAID sparing effect. We concluded that there exists an inflammatory subtype of disease where methotrexate can be beneficial.
厚皮性骨膜病是一种罕见的遗传性疾病，其特征为皮肤增厚、杵状指和骨膜炎。其发病机制尚未完全明确，且对于其症状尚无经证实有效的治疗方法。虽然据报道20% - 40%的病例存在关节炎，但大多数本质上是非炎性的，通常使用类固醇或非甾体抗炎药进行对症治疗。我们报告了两例厚皮性骨膜病合并炎性关节炎且炎症标志物升高的病例，这两例患者接受了为期6周的逐渐减量泼尼松龙治疗，并像类风湿关节炎患者一样长期服用低剂量甲氨蝶呤进行维持治疗，随访2年。在这两例病例中，甲氨蝶呤耐受性良好，有助于维持症状改善并减缓疾病进展，同时具有显著的减少类固醇和非甾体抗炎药用量的效果。我们得出结论，该疾病存在一种炎性亚型，在此亚型中甲氨蝶呤可能有益。
