特发性肺纤维化76例临床分析

Analysis of clinical characteristics of seventy-six patients with idiopathic pulmonary fibrosis

关键词:  特发性肺纤维化  临床表现  治疗

Key words:  idiopathic pulmonary fibrosis  clinical manifestation  treatments

摘要:

［目的］ 通过总结特发性肺纤维化（IPF）的临床特点，提高对特发性肺纤维化的诊断及治疗的认识。［方法］ 回顾性分析2002年1月~2008年12月大连医科大学附属第二医院 76例特发性肺纤维化患者的临床资料。［结果］ 76例患者均有干咳及进行性呼吸困难。胸部CT表现为磨玻璃样、网状、结节、条索状及蜂窝状阴影。42例肺功能检查患者中，限制性通气功能障碍25例，混合性通气功能障碍9例。动脉血气分析示35例低氧血症， 30例1型呼衰。死亡9例。［结论］ 对有慢性咳嗽和进行性呼吸困难的患者及时进行胸部CT检查、肺功能和动脉血气分析，可对本病做出正确诊断，及早糖皮质激素治疗效果较好。大部分患者临床症状有所缓解，但肺间质纤维化影像学表现并未得到明显改善。

Abstract:

［Objective］ To summarize the clinical characteristics of idiopathic pulmonary fibrosis(IPF) to build up knowledge for early diagnosis. ［Methods］ Seventy-six IPF patients, diagnosed between Jan. 2002 and Dec. 2008 in the hospital were reviewed retrospectively. ［Results］ Most IPF patients had an insidious onset of progressive dyspnea and non-productive cough. The main manifestations of chest CT were reticular, fine nodul and honeycomb formations. Among 42 patients, who took pulmonary function test, 25 patients showed restrictive impairment, and 9 of them had mixed ventilation disturbances. Thirty-five of the 76 patients were revealed hypoxemia on the resting arterial blood gas and 30 of them were with type I respiratory failure. Only 9 deaths occurred in this study. ［Conclusions］ Chest CT, pulmonary function test and blood gas analysis should be taken early in IPF patients with progressive dyspnea and non-productive cough in order for them to obtain timely diagnosis and treatment. Therapy with glucocorticoid may be effective in the improvement of symptoms, step by step, in most of the patients. However, the chest CT of the patients was not obviously getting better.