To date, as many as 45% of all deaths in the developed world are related to pathological tissue remodeling. Chronic lung diseases (CLD) are the 3rd leading cause of death worldwide. Among CLD, Idiopathic Pulmonary Fibrosis (IPF) is a chronic and lethal fibroproliferative disease of the lung, with unknown etiology. IPF is characterized by progressive fibrosis and irreversible loss of lung function, with a median survival, or time to lung transplantation, of about 3 years after diagnosis. In many organs, innate and adaptive immunity contributes to fibrogenesis. Rising evidence shows that abnormal immune responses are implicated in IPF pathophysiology.