Primary interstitial pneumopathies

Abstract

Primitive interstitial pneumopathies are only a temporary classification. They seem to be due to an evolution for the worse of a "diffuse alveolar damage" of unknown origin. Evolution towards pulmonary fibrosis and respiratory insufficiency is their common risk. Etiopathogenic hypotheses would favour the immunological etiology. A diagnosis of primitive interstitial pneumopathy is reached by elmination when no known cause can be found. Liebow's classification remains valuable for pathology but is debatable where anatomo-clinical correlations, etiological hypotheses and the eventual effect of treatment are concerned. Today these primitive forms still represent 40% of the interstitial lung diseases, the causes of which are progressively discovered.