Mydlak Anna, Sołdacki Dariusz, Foroncewicz Bartosz, Stopa Zygmunt, Powała Agnieszka, Budlewski Tadeusz, Pączek Leszek, Mucha Krzysztof
Department of Cranio-Maxillofacial Surgery, Oral Surgery and Implantology, Medical University of Warsaw Department of Immunology, Transplantology and Internal Diseases, Medical University of Warsaw Department of Pathology, Medical University of Warsaw Nuclear Medicine Unit, Department of Diagnostic Radiology, Central Clinical Hospital Ministry of Internal Affairs Institute of Biochemistry and Biophysics, Polish Academy of Sciences, Warsaw, Poland.
Medicine (Baltimore). 2017 Oct;96(43):e8360. doi: 10.1097/MD.0000000000008360.
Relapsing polychondritis (RP) is a multisystemic, progressive disease of unknown etiology characterized by recurrent inflammation and progressive cartilage destruction. It can involve all types of cartilage including ears and nose, tracheobronchial tree, joints, and any other tissue rich in proteoglycans such as heart, eyes, and blood vessels. Recurrent chondritis can be life-threatening if the respiratory tract, heart valves, or blood vessels are affected. To date there is no data in the literature on the post solid organ transplantation RP.
We present a 59-year-old male liver transplant recipient with primary sclerosing cholangitis who developed RP of the earlobes and nose despite post-transplant immunosuppression.
Based on the clinical criteria, scintigraphy and biopsy from the left auricle his condition was diagnosed as RP.
Pulses of methylprednisolone followed by high-dose oral steroids along with azathioprine were administered.
Such therapy diminished local cartilage inflammation, improved patient's general condition and the laboratory results. Significant loss of ear cartilage and characteristic "saddlenose" were observed after remission of acute symptoms. The control scintigraphy proved very good treatment response.
To the best of our knowledge this is the first report on the RP in liver transplant recipient. Based on our patient presentation, we suggest that RP should be suspected in any transplant recipient with cartilage inflammation, and that the Michet's clinical criteria and scintigraphy seem to be the best diagnostic tools for solid organ transplant recipients suspected of RP.
复发性多软骨炎(RP)是一种病因不明的多系统进行性疾病,其特征为反复炎症和进行性软骨破坏。它可累及所有类型的软骨,包括耳朵和鼻子、气管支气管树、关节以及任何其他富含蛋白聚糖的组织,如心脏、眼睛和血管。如果呼吸道、心脏瓣膜或血管受到影响,反复的软骨炎可能会危及生命。迄今为止,文献中尚无关于实体器官移植后发生RP的数据。
我们报告一例59岁男性肝移植受者,患有原发性硬化性胆管炎,尽管移植后进行了免疫抑制,但仍出现了耳垂和鼻子的RP。
根据临床标准、闪烁扫描以及左耳廓活检,其病情被诊断为RP。
给予甲泼尼龙冲击治疗,随后使用高剂量口服类固醇以及硫唑嘌呤。
这种治疗减轻了局部软骨炎症,改善了患者的一般状况和实验室检查结果。急性症状缓解后,观察到耳部软骨明显缺失以及典型的“鞍鼻”。对照闪烁扫描显示治疗反应非常好。
据我们所知,这是首例关于肝移植受者发生RP的报告。根据我们的患者情况,我们建议对于任何出现软骨炎症的移植受者都应怀疑患有RP,并且米歇特临床标准和闪烁扫描似乎是疑似RP的实体器官移植受者的最佳诊断工具。