特发性肺纤维化合并慢性支气管炎2例报告并文献复习

摘要

目的：提高对特发性肺纤维化合并慢性支气管炎的临床认识，探讨特发性肺纤维化发病机制。方法：回顾2例特发性肺纤维化合并慢性支气管炎的临床表现、影像学特点及诊治经过，结合有关文献进行回顾性分析。结果：2例均为老年患者，男女各1例。均在慢性支气管炎的基础上出现呼吸困难加重，胸部高分辨 CT（chest high resolution，HRCT）均表现为普通型间质性肺炎特点，并排除其他已知原因的间质性肺疾病，诊断特发性肺纤维化成立。两例均接受了糖皮质激素联合 N-乙酰半胱氨酸的治疗方案，随访时间1～2年，病情长期稳定，糖皮质激素均减量至10 mg ／d 长期维持。其中病例1最近1次住院中发现病情有所加重。结论：特发性肺纤维化患者病情呈不可逆进展，治疗无特效药物，预后不良，可酌情选用糖皮质激素联合 N-乙酰半胱氨酸的方案。若合并慢性支气管炎，病情更复杂，容易导致预后不佳。%Objective:To improve the clinical understanding of idiopathic pulmonary fibrosis (IPF)combined with chronic bronchitis and investigate pathogenesis of IPF.Methods:To review the clinical manifestations,imaging features and diagnosis and treat-ment of 2 cases of IPF combined with chronic bronchitis,and review the relevant literatures.Results:Two patients were more than 60 years old.One was female,and the other male.They had a history of chronic bronchitis,and felt aggravated dyspnea.Chest high resolu-tion CT (HRCT)showed the feature of usual interstitial pneumonia,and other known causes of interstitial lung disease were excluded. So diagnosis of IPF was established.Two patients received the treatment of glucocorticoid combined with N-cysteine,the follow-up peri-od was 1 + to 2 + years with the long-term stability of the disease,and the maintenance dose of glucocorticoids is 10 mg /d.In addition the first patient was found to be aggravated during the last hospitalization.Conclusion:The progression of IPF was irreversible and the prognosis was not good.The disease has no specific treatment drugs.We can choose the treatment of glucocorticoid combined with N-cysteine.If IPF was combined with chronic bronchitis,the disease is more complex and it will lead to poor prognosis of patients.