以呼吸系统症状为主的复发性多软骨炎患者的影像学、纤支镜及肺功能特点

The Characteristics of Imaging,Fiber-bronchoscope and Pulmonary Function Testing of Relapsing Polychondritis Patients with Respiratory Symptoms

目的研究以呼吸系统症状为主的复发性多软骨炎(relapsing polychondritis,RP)患者临床表现、影像学、纤支镜、肺功能等特点。方法回顾性分析四川大学华西医院2007年5月至2017年5月住院患者中明确诊断为RP患者33例的临床资料,收集患者临床表现、实验室检查、影像学(CT检查)、纤支镜及肺功能等检查资料。根据是否以呼吸系统症状为主将患者分为呼吸组(21例)和非呼吸组(12例),并对两组患者数据进行统计学分析。结果 33例患者中,呼吸系统受累最常见,占63.6%,其余依次为耳部(48.5%)、关节(30.3%)、皮肤(15.2%)、鼻部(12.1%)、眼部(12.1%)受累。呼吸组患者主要临床表现为咳嗽、咳痰、气促、发热,非呼吸组主要以耳、鼻、关节受累为主,表现为受累部位肿胀、疼痛。CT显示,呼吸组中有13例患者(61.9%)气管及主支气管壁增厚、钙化,管腔变窄,非呼吸组患者中仅3例患者(25.0%)有支气管管壁增厚,但无气道狭窄;纤支镜结果显示,呼吸组患者86.7%(13/15)有异常发现(包括气管软骨环结构消失、黏膜肿胀、肥厚、气管管腔狭窄、塌陷),非呼吸组患者中5例检查者均无气道管腔异常。肺功能检查结果中,呼吸组患者一秒用力呼气容积(FEV1)、[FEV1/用力肺活量(FVC)]%和峰值呼气流速(PEF)低于非呼吸组患者,差异均有统计学意义(P<0.05)。结论以呼吸系统症状为主的RP缺乏特异性的确诊手段,但结合CT、纤支镜、肺功能等检查手段可提供呼吸系统早期受累的证据。

Objective To analyze the characteristics of clinical manifestation, imaging, fiber-bronchoscope finding and pulmonary function testing in the patients of relapsing polychodriti with respiratory symptoms. Methods The clinical data of 33 patients with RP in West China Hospital of Sichuan University from May 2007 to May 2017 were analyzed retrospectively. The patients were divided into respiratory group （21 cases） and non-respiratory group （12 cases） based on whether the respiratory symptoms were main involvement, and the data of the two groups were statistically analyzed. Results Among the 33 patients, the most vulnerable system was respiratory system, accounting for 63.6%. The other involvement areas were as follows： ears 48. 5%, joints 30.3%, skin 15.2%, noses and eyes 12.1 %respectively. The chief complaints in the respiratory group were cough, polypnea, and fever. The major manifestations of the non-respiratory group were pain and swelling of ears and nose, joints. CT showed that the wall of trachea and main bronchus were thickened, calcified, and narrowed in 13 cases （61.9%） of the respiratory group, while 3 patients （25.0%） found thickened bronchus wall without bronchostenosis in non- respiratory group . Bronchoscopy showed that 86.7% （13/15） of the patients were found airway abnormal in the respiratory group, and none of the patients in the non-respiratory group had. In the test of pulmonary function, the respiratory group had lower one second forced expiratory volume （FEV1）, [FEV1/ forced vital capacity （FVC）] and peak expiratory flow （PEF）, and all the differences were significant （P（0.05）. Conclusion CT, broncho- scopy, and pulmonary function tests could provide early evidence to diagnosis of relapsing polychondritis, which is lack of specificity of diagnosis, especially respiratory system symptom onset.