Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes.
Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes.
复制标题
DOI：
10.3390/medsci6040110
复制
发表时间：
2018-11-29
期刊：
影响因子：
--
通讯作者：
Soriano JB
中科院分区：
文献类型：
--
作者：
Sauleda J;Núñez B;Sala E;Soriano JB
Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is characterized by a chronic, progressive, fibrotic interstitial lung disease of unknown cause that occurs primarily in older adults. Its prevalence and incidence have appeared to be increasing over the last decades. Despite its unknown nature, several genetic and environmental factors have been associated with IPF. Moreover, its natural history is variable, but could change depending on the currently suggested phenotypes: rapidly progressive IPF, familial, combined pulmonary fibrosis and emphysema, pulmonary hypertension, and that associated with connective tissue diseases. Early recognition and accurate staging are likely to improve outcomes and induce a prompt initiation of antifibrotics therapy. Treatment is expected to be more effective in the early stages of the disease, while developments in treatment aim to improve the current median survival of 3–4 years after diagnosis.
