特发性肺纤维化的治疗进展

Advances in therapy of idiopathic pulmonary fibrosis

特发性肺纤维化是一种慢性、弥散性肺间质病变,以普通型间质性肺炎为特征性组织学表现。特发性肺纤维化以进行性肺损伤、炎症、肺组织纤维化为特征。糖皮质激素和免疫抑制剂/细胞毒性药物临床效果欠佳,提示炎症并未在肺纤维化中起重要的病理生理作用,研究发现肺泡上皮损伤可以直接导致肺纤维化。目前尚无有效的治疗方案能取得肯定的疗效。因此,新的治疗措施引发了研究者浓厚的兴趣。然而特发性肺纤维化的研究进展缓慢,预后很差,诊断后中期生存率小于3年。本文主要介绍特发性肺纤维化在治疗方面的进展。

Idiopathic pulmonary fibrosis （IPF）is a chronic, diffuse interstitial pulmonary disease associated with a characteristic histologic appearance of usual interstitial pneumonia. IPF is characterized by progressive injury, inflammation, and fibrosis of the lung parenchyma. The clinical ineffectiveness of corticosteroids and immunosuppressives/cytotoxics suggests that inflammation does not play a prominent pathophysiologic role. Studies show that alveolar epithelial injuries directly result in fibrosis. There is no effective therapy with proven and unequivocal benefits currently available. For this reason, novel therapeutic modalities are of strong interests. However, IPF generally progresses relentlessly and carries the poorest prognosis of the chronic IPF,with a median survival of less than 3 years after diagnosis. This article mainly presents the advances in the therapy of IPF.