• Harefuah · Jul 2015

    Review

    [Novel therapies for idiopathic pulmonary fibrosis].

    • Ori Wand and Mordechai R Kremer.
    • Harefuah. 2015 Jul 1;154(7):446-50, 469, 468.

    AbstractIdiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown cause. The clinical course is unpredictable, but the disease is usually progressive with a median survival of 2-5 years as a result of advanced respiratory failure. The current hypothesis of the disease mechanism is recurrent injury to the respiratory epithelium which leads to an uncontrolled wound healing process resulting in fibrosis rather than repair. Despite better understanding of the pathogenesis, there is no effective therapy for the disease. In this review, we present the positive results of recently published clinical trials regarding therapy for IPF, with emphasis on pirfenidone and nintedanib.

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