Idiopathic pulmonary fibrosis Modest progress nearly 150 years after the condition was first described

摘要

In 1868, Flint described a condition called "chronic pneumonitis" and noted the bulbous appearance of one patient's fingertips, later known as finger clubbing. This was probably the first recorded case of idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis is a devastating form of interstitial lung disease of unknown cause. With a median survival of three years after diagnosis, it carries a prognosis worse than many cancers. At a conservative estimate, there are 5000 new cases each year in the United Kingdom, and at least 14 000 new patients diagnosed annually in the United States. Median age of onset is 70 years and about two thirds of patients are smokers. Once thought of as a chronic inflammatory condition, the disease is more likely to be a fibrotic response driven by abnormally activated alveolar epithelial cells.