复发性多软骨炎累及呼吸道1例并文献复习

发布时间：2018-03-24 03:10

本文选题：复发性多软骨炎　切入点：呼吸道受累　出处：《广西医科大学》2015年硕士论文

【摘要】：目的：探讨复发性多软骨炎累及呼吸道的临床特征,以提高认识,早期发现并诊断,及早综合性治疗,改善预后。方法：对在广西医科大学一附院住院确诊的1例复发性多软骨炎累及呼吸道患者的临床资料进行总结分析并结合文献复习。结果：1.本例患者21岁男性,以反复发热、胸痛、气促为主要首发临床症状,病程中出现声音嘶哑、听力下降、鞍鼻、双眼视物模糊、皮疹,白细胞、C反应蛋白、血沉等指标升高,肺部CT示气管、支气管钙化,肺功能提示阻塞性通气功能障碍,纤维支气管镜见气管上段狭窄,给予激素联合免疫抑制剂治疗后症状改善。2.文献复习结果：从PUBMED等多个数据库检索从2005年1月年至2015年1月国内外对RP患者累及呼吸道个案报道的例数,初步检索文献74篇,依据诊断标准39篇符合,共报道50例,均有较完整的病史及临床资料。其中男性29例,女性21例,年龄最小20岁,最大83岁,平均50.7岁。呼吸系统主要临床表现为咳嗽、咳痰、呼吸困难、声嘶及肺部哮鸣音。48例行肺CT检查,多表现为气管支气管管壁增厚,管腔狭窄。28例行肺功能检查,以阻塞性通气功能障碍为主。只有10例行支气管黏膜活检。曾误诊为哮喘17例,支气管炎12例,COPD5例。单纯予激素治疗好转共14例,需加用免疫抑制剂22例,而需介入或无创机械通气共10例。只有部分患者进行随访,1年后随访2例,2年后随访2例,5年后随访1例,均无复发。但1例患者半年后死于呼吸衰竭。结论：1.RP累及呼吸道患者主要症状为咳嗽、呼吸困难等,伴耳、鼻受累等肺外表现；2.肺功能以阻塞性通气功能障碍为主要表现,胸部影像学、支气管镜检查可明确气道受累情况；3.常易误诊为哮喘、支气管炎等；4.全身治疗以激素为主,局部治疗包括手术介入、机械通气,预后不佳。
[Abstract]:Objective: to investigate the clinical features of recurrent polychondrositis involving respiratory tract in order to improve the understanding, early detection and diagnosis, and early comprehensive treatment. Methods: to summarize and analyze the clinical data of a case of recurrent polychondrositis involving respiratory tract diagnosed in the first affiliated Hospital of Guangxi Medical University and review the literature. Results: 1. This case was 21 years old male with repeated fever. Chest pain and shortness of breath were the main clinical symptoms. In the course of the disease, hoarseness, hearing loss, saddle nose, blur of binocular vision, rash, leukocyte C reactive protein, erythrocyte sedimentation rate and so on were increased. Lung CT showed calcification of trachea and bronchus. Pulmonary function suggests obstructive ventilation dysfunction, fibrobronchoscopy shows upper trachea stenosis, Results of literature review: from January 2005 to January 2015, the number of cases reported on respiratory tract involvement in RP patients from January 2005 to January 2015 was retrieved from literature review, and 74 articles were preliminarily searched. According to 39 diagnostic criteria, 50 cases were reported with complete history and clinical data. Among them, 29 cases were male, 21 cases were female, the youngest was 20 years old, the maximum was 83 years old, the average was 50.7 years old. The main clinical manifestations of respiratory system were cough and expectoration. Pulmonary CT examination was performed in 48 cases with dyspnea, hoarseness and wheezing of lung. The main manifestations were thickening of tracheobronchial wall and stenosis of lumen in 28 cases. Only 10 cases of bronchial mucosal biopsy were misdiagnosed as asthma (17 cases), bronchitis (12 cases) and cod (5 cases). Only some patients were followed up, 2 cases after 1 year, 2 cases after 2 years, 1 case after 5 years. No recurrence occurred, but one patient died of respiratory failure half a year later. Conclusion 1. The main symptoms of respiratory tract involvement with RP are cough, dyspnea and other extrapulmonary manifestations. 2. Obstructive ventilation dysfunction is the main manifestation of pulmonary function. Chest imaging and bronchoscopy can identify airway involvement 3.It is often misdiagnosed as asthma, bronchitis and so on. 4. Hormone is the main treatment for the whole body. Local treatment includes surgical intervention, mechanical ventilation and poor prognosis.
【学位授予单位】：广西医科大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R593.2

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