Table 1.

Potential Therapies for Fibrotic Interstitial Lung Disease

Disease	Medication	Dose	Mechanism of Action
Idiopathic pulmonary fibrosis	Nintedanib	150 mg bid	Kinase inhibitor; inhibition of VEGFR 1-3, FGFR 1-3, PDGFR α and β
	Pirfenidone	801 mg tid	Possible inhibition of transforming growth factor-β and tumor necrosis factor-α
Chronic hypersensitivity pneumonitis	Prednisone	0.5-1 mg/kg/d up to 60 mg/d, then tapered to lowest effective dose	Inhibition of multiple inflammatory cytokines
Connective tissue disease-associated	Prednisone	0.5-1 mg/kg/d up to 60 mg/d Doses > 20 mg/d are not advised in SSc	Inhibition of multiple inflammatory cytokines
	Azathioprine	1.5-2 mg/kg/d	Purine antagonist; inhibition of cellular and humoral immunity
	Cyclophosphamide	1-2 mg/kg/d po or 500-1,000 mg IV every 4 wk	Alkylating agent; crosslinks DNA
	Mycophenolate mofetil	1,000 mg bid up to 3,000 mg/d	Inhibition of B- and T-lymphocyte proliferation
	Rituximab	1,000 mg IV, repeat in 2 wk	Monoclonal antibody which binds and depletes B-lymphocyte CD20
	Tacrolimus	1 mg bid titrated by 1 mg based on trough levels	Calcineurin inhibitor; inhibits activation of T-lymphocytes

All these medications are given by mouth unless otherwise indicated.

FGFR = fibroblast growth factor receptor; PDGFR = platelet-derived growth factor receptor; SSc = systemic sclerosis; VEGFR = vascular endothelial growth factor receptor.