Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) caused by an inhalational exposure to low-molecular weight compounds, which occurs in susceptible individuals. Hypersensitivity pneumonitis may present in two forms: acute, predominantly inflammatory HP (non-fibrotic HP), and chronic or fibrotic HP (fibrotic HP). 1 Some patients will experience a self-limiting acute HP course, whereas others, given sufficient time and exposure, will progress from non-fibrotic to fibrotic disease, and still other patients may present with established fibrotic HP without a clear acute episode of exposure. This suggests that the pathophysiology of HP is contributed by complex pathways of antigen exposure, aberrant immunological mechanisms, and genetic predispositions.
| EpidemiologyReported estimates of the incidence of HP vary across populations, with 1-3 per 100,000 people per year (including children) in Denmark, 2 11.5 per 100,000 people over 65 years per year in the USA, 3 and up to 30 per 100,000 people per year in New Mexico. 4 HP is the third most common ILD after idiopathic pulmonary fibrosis (IPF) and connective tissue disease-related interstitial lung disease (CTD-ILD). 5 The estimated prevalence of HP is higher in at-risk