Nouveau concept d'IPAF (pneumopathie interstitielle avec manifestations auto-immunes)

Interstitial lung disease (ILD) often complicates connective tissue disease. Sometimes, patients with interstitial lung disease have auto-immune features, but fail to meet diagnostic criteria for a specific connective tissue disease. The management of this group of patients is difficult, as the border between idiopathic ILD and connective tissue disease is unclear. Multidisciplinary discussion involving a rheumatologist experienced in systemic diseases is useful when a connective tissue disease is suspected. Several classifications have been proposed to standardize this group of patients, but none has been validated. Recently, an international task force of the American Thoracic Society and the European Respiratory Society proposed a new terminology and consensus criteria defining interstitial pneumonia with auto-immune features (IPAF) in order to standardize and better characterize this group of patients. In this monograph, we will study the new concept of IPAF, after having detailed the previous concepts. We will then describe the recent cohorts resulting from this new classification and future perspectives that remain to be explored further.