Aims : to describe the magnitude of the problem of progressive pulmonary fibrosis (PPF); to discuss the diagnostic tools to detect or predict PPF; to explore some of the putative pathogenic mechanisms that may be involved in the progression of fibrotic lung disorder; to discuss the therapeutic approach, covering immunosuppression and antifibrotic drugs, antifibrotic drugs alone, nonpharmacological support, and when to start and stop this all.