ABSTRACT
Pulmonary fibrosis (PF) is a heterogeneous clinical syndrome that represents the end-stage of chronic interstitial lung diseases. Dozens of different occupational, environmental, immune and genetic risk factors have been associated with PF, and through the past several decades, risk factor exposures have been the driving force in the diagnostic classification of PF, thus in the current paradigm, there are dozens of different diagnoses of pulmonary fibrosis. This emphasis on distinction has focused much attention on the most common form of this syndrome (Idiopathic Pulmonary Fibrosis, IPF), which comprises only 20 of PF patients. Today there are2 modestly effective FDA-approved treatments for IPF;however, for the 80 of PF patients with other diagnoses, there are no known effective treatments.