ABSTRACT

The idiopathic interstitial pneumonias (IIP) are a heterogeneous group of pul-

monary diseases that share a general though not universal propensity for the

insidious development of chronic and progressive lung injury. Acute interstitial

pneumonia (AIP) is unique among the IIP with its rapid onset, early respiratory

failure, and high initial case fatality ratio. Survivors of the initial insult have a

more favorable long-term prognosis than the most common of the IIP, idiopathic

pulmonary fibrosis (IPF). A rare disease, our current knowledge of AIP is limited

and is derived mostly from observational studies of small case series.