Young R C, Sahetya G K, Hassan S N, Cobb W B, Kumar B, Facen H T
J Natl Med Assoc. 1980 Oct;72(10):965-72.
Emphysema, chronic bronchitis, asthma, and cystic fibrosis are often cited as examples of chronic airways obstruction, while sarcoidosis is typically restrictive. Approximately 15 percent of sarcoidosis patients, however, have airways obstruction clinically characterized by wheezing with granulomatous involvement of airways. Since the majority have Stage IV disease by chest radiograph, their lungs usually have honey-combining with pulmonary fibrosis, adhesions, cavities, and mediastinal distortion.Patients had a mixed ventilatory defect, but obstruction of large airways was present as shown by decreased specific airways conductances. Small airways obstruction was also present as shown by low instantaneous flows at the terminal portion of the maximum expiratory flow-volume curve and diminished helium response of this curve. The closing volume, however, was not very sensitive. Radioactive xenon washout from ventilation lung scans and N2 washout from the lungs were prolonged in patients with worse disease.The authors conclude that the obstructive type of physiologic pattern is more frequent than recognized in sarcoidosis, which like that of cystic fibrosis has some restrictive element and is characterized by poor reversibility to bronchodilators. A trial period of beclomethasone dipropionate aerosol was not helpful in two patients. Relief of this distressing airways obstruction continues to pose a challenging problem in management.
肺气肿、慢性支气管炎、哮喘和囊性纤维化常被引为慢性气道阻塞的例子,而结节病通常是限制性的。然而,约15%的结节病患者存在气道阻塞,临床上表现为喘息,气道有肉芽肿累及。由于大多数患者胸部X线片显示为IV期疾病,其肺部通常有蜂窝状改变,并伴有肺纤维化、粘连、空洞和纵隔变形。患者存在混合性通气缺陷,但如气道比传导率降低所示,存在大气道阻塞。如最大呼气流量-容积曲线终末部分的瞬间流量降低及该曲线的氦反应减弱所示,也存在小气道阻塞。然而,闭合气量不太敏感。病情较重的患者通气肺扫描的放射性氙洗脱和肺部的氮气洗脱时间延长。作者得出结论,结节病中阻塞型生理模式比人们认识到的更为常见,它与囊性纤维化一样有一些限制性因素,其特点是对支气管扩张剂的反应性差。两名患者使用丙酸倍氯米松气雾剂的试验期并无帮助。缓解这种令人痛苦的气道阻塞在治疗中仍然是一个具有挑战性的问题。