Bouros Demosthenes, Antoniou Katerina M, Tzouvelekis Argyris, Siafakas Nikolaos M
Democritus University of Thrace, Department of Pneumonology, Medical School, Alexandroupolis, Greece.
Expert Opin Biol Ther. 2006 Oct;6(10):1051-60. doi: 10.1517/14712598.6.10.1051.
Idiopathic pulmonary fibrosis (IPF) represents a particularly aggressive disease, the aetiology of which still remains unknown. The natural history of the disease often leads to respiratory failure and death, with a mortality rate greater than some cancers. To date, no management approach has proven to be efficacious for the treatment of this disease. IPF has been characterised as an 'epithelial-fibroblastic disorder', characterised by abnormal wound healing with excessive fibrosis and minimal inflammation. These emerging data have focused attention on antifibrotic drugs. Interferon-gamma1b (IFN-gamma1b) has recently been proposed as a promising candidate for the treatment of IPF. The reason for this is that IFN-gamma1b has the ability to modulate the Th1/Th2 imbalance and to suppress fibroblast activation. The view that IPF is untreatable at present requires reconsideration, as improved survival has been suggested in three controlled trials of IFN-gamma1b in IPF therapy.
特发性肺纤维化（IPF）是一种侵袭性很强的疾病，其病因至今仍不明。该病的自然病程常导致呼吸衰竭和死亡，死亡率高于某些癌症。迄今为止，尚无治疗方法被证明对该病有效。IPF被认为是一种“上皮-成纤维细胞紊乱”，其特征是伤口愈合异常，伴有过度纤维化和轻微炎症。这些新出现的数据使人们将注意力集中在抗纤维化药物上。γ-干扰素1b（IFN-γ1b）最近被认为是治疗IPF的一个有前景的候选药物。原因是IFN-γ1b能够调节Th1/Th2失衡并抑制成纤维细胞活化。鉴于在三项IFN-γ1b治疗IPF的对照试验中提示生存率有所提高，目前认为IPF无法治疗的观点需要重新审视。
