The term "acute interstitial pneumonia" (AIP) describes an idiopathic clini
copathological condition, characterized clinically by an interstitial lung
disease causing rapid onset of respiratory failure, which is distinguishabl
e from the other more chronic forms of interstitial pneumonia, It is synony
mous with Hamman-Rich syndrome, occurring in patients without pre-existing
lung disease.
The histopathological findings are those of diffuse alveolar damage. AIP ra
diologically and physiologically resembles acute respiratory distress syndr
ome (ARDS) and is considered to represent the small subset of patients with
idiopathic ARDS. It is frequently confused with other clinical entities ch
aracterized by rapidly progressive interstitial pneumonia, especially secon
dary acute interstitial pneumonia, acute exacerbations and accelerated form
s of cryptogenic fibrosing alveolitis. Furthermore, many authors use the ab
ove terms, both erroneously and interchangeably. It has a grave prognosis w
ith >70% mortality in 3 months, despite mechanical ventilation.
This review aims to clarify the relative clinical and pathological issues a
nd terminology.