复发性多软骨炎呼吸道受累的特点

目的研究复发性多软骨炎(RP)呼吸道受累的特点。方法回顾性分析呼吸道受累和未受累的RP患者临床表现、呼吸功能试验、胸部CT、支气管镜检查的特点及其间相关性。结果38例呼吸道受累患者中男性20例、女性18例,发病年龄45岁±11岁(27~71岁),症状有咳嗽、咯痰、声嘶、憋气、喘息、呼吸困难等。18例行呼吸功能试验,其中阻塞性通气功能障碍10例,混合性通气功能障碍7例;与无呼吸道受累的RP患者相比,呼吸道受累者FEV1(1·4L±0·23Lvs2·5L±0·09L,P=0·001)、FEV1/FVC(46·0±4·86vs83·7±2·24,P=0·000)、PEF(3·3L/s±0·67L/svs6·9L/s±0·52L/s,P=0·006)、FEF50/FIF50(0·3±0·08vs1·3±0·51,P=0·010)、MMEF25~75(0·4L/s±0·18L/svs2·8L/s±0·73L/s,P=0·010)均明显下降;呼吸流速容量环显示呼气峰流速下降,呼气相平台形成。27例行胸部CT检查,气道狭窄11例、气管壁增厚8例。23例行支气管镜检查,黏膜炎症16例、软骨环消失6例、气道塌陷7例、正气道狭窄15例、左右主支气管狭窄分别为13和12例。结论呼吸功能试验是一项能早期发现RP呼吸道受累的敏感性高的检查,胸部CT和支气管镜检查对评价RP患者的气道受累情况有一定意义。

Characteristics of airway involvement in relapsing polychondritis

OBJECTIVE: To investigate the characteristics of airway involvement in relapsing polychondritis (RP). METHODS: The clinical data, including clinical manifestations, respiratory function test, computerized tomography (CT), and bronchoscopy of 38 out of the 56 RP patients who had airway involvement, 20 males and 18 females, with the mean onset age of 45 +/- 11 (27 - 71), and 3 RP patients without airway involvement were retrospectively analyzed. Three patients out of the 16 RP patients who did not have respiratory involvement but underwent respiratory function test, CT, and bronchoscopy were used as controls. RESULTS: The symptoms of airway involvement included cough, expectoration, hoarseness, feeling of suffocation, asthma, and dyspnea. Obstructive disturbance of ventilation was found in 10 and mixed disturbance of ventilation was seen in 2 of the 18 RP patients with airway involvement. The forced expiratory volume in one second, ratio of forced expiratory volume in one second to forced vital capacity, peak expiratory flow, FEF50/FIF50, and maximal mild-expiratory flow of the patients with airway involvement were 1.4 L +/- 0.23 L, 46.0 +/- 4.86, 3.3 L/s +/- 0.67 L/s, 0.3 +/- 0.08, and 0.4 L/s +/- 0.18 L/s respectively, all significantly lower than those of the RP patients without airway involvement (2.5 L +/- 0.09 L, 83.7 +/- 2.24, 6.9 L/s +/- 0.52 L/s, 1.3 +/- 0.51, and 2.8 L/s +/- 0.73 L/s, all P = 0.01). Flow volume loop showed remarkable decrease of PEF and formation of a plateau in the expiratory phase in the RP patients with airway involvement. CT performed in 27 RP patients with airway involvement showed trachea stenosis in 11, and thickened airway wall in 8 of them. Bronchoscopy performed in 23 patients with airway involvement showed inflammation in 16, destruction of tracheobronchial cartilage in 6, collapsed tracheobronchial wall in 7, tracheal stenosis in 15, left major bronchial stenosis in 13, and right major bronchial stenosis in 12 of them. CONCLUSION: Respiratory function test is sensitive in early detection of airway involvement in RP. Bronchoscopy and CT are useful in evaluation of the severity of airway involvement in patients with RP.