Among the interstitial lung diseases (ILD), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis and fibrotic connective tissue disease related ILD are associated with a worse prognosis with death occurring both from respiratory failure and serious associated co-morbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF. Although chronic hypersensitivity pneumonitis and connective tissue disease related-ILD may be associated with an inflammatory component, the evidence for the use of immunosuppressive agents in their treatment is largely limited to retrospective studies. The lack of benefit of immunosuppressive therapy in advanced fibrosis argues for rigorous clinical trials using anti-fibrotic therapies in these types of ILD as well. Patients with fibrotic ILD may benefit from identification and management of associated co-morbid conditions such as pulmonary hypertension, gastroesophageal reflux and obstructive sleep apnea, which may improve the quality of life, and in some cases, survival in affected individuals. Lung transplant evaluation should occur early in patients with IPF and those with other forms of fibrotic ILD as early evaluation may optimize post- transplantation outcomes.