Idiopathic pulmonary fibrosis (complications (pulmonary HTN, acute…
Idiopathic pulmonary fibrosis
Specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia
exposure to an inciting agent (eg, smoke, environmental pollutants, environmental dust, viral infections, gastroesophageal reflux disease, chronic aspiration) in a susceptible host may lead to the initial alveolar epithelial damage.
Epithelial-fibroblastic disease, in which unknown endogenous or environmental stimuli disrupt the homeostasis of alveolar epithelial cells, resulting in diffuse epithelial cell activation and aberrant epithelial cell repair.
10.7 per 100,000 person years
mean surivival 2-5y
associated systemic symptoms
fine bibasal crackles
acute exacerbation of pulmonary fibrosis
acute coronary syndrome
peripheral reticular opacities
pulmonary function tests