Approved Treatments for Pulmonary Fibrosis

Cytoxan (cyclophosphamide) is a chemotherapy agent that can help suppress inflammation and has been used to treat certain forms of pulmonary fibrosis. It works by stopping or slowing the growth of certain types of cancer cells. It usually is taken orally but also may be administered intravenously.

Esbriet (pirfenidone) is an oral treatment for idiopathic pulmonary fibrosis (IPF) that is known to decrease inflammation by reducing the synthesis of a pro-inflammatory molecule called transforming growth factor beta (TGF-beta). This reduces excessive collagen production and secretion of other inflammatory mediators such as tumor necrosis factor alpha and interleukin 1 beta.

Imuran (azathioprine) is an immunosuppressant, or medication that suppresses the immune system. It is used together with other medications to prevent rejection of transplants. It also is used to treat inflammatory diseases, connective tissue diseases, and autoimmune diseases when other medications and treatments do not work.

Mycophenolate mofetil (sold under the brand names CellCept and Myfortic) is an oral immunomodulatory agent that inhibits the proliferation of lymphocytes. Apart from its uses in preventing transplant rejection, it is also an anti-inflammatory and an anti-proliferative agent because it acts on some growth factors, including TGF-beta, a type of cytokine that controls proliferation, cellular differentiation, and other functions in cells.

Ofev (nintedanib) is an anti-fibrotic medicine that inhibits tyrosine kinase, an enzyme essential for signal transduction pathways that trigger growth factors like vascular endothelial growth factor receptor, fibroblast growth factor receptor, and platelet-derived growth factor receptor. These are produced in excess in idiopathic PF (IPF) patients. By inhibiting tyrosine kinase and blocking these pro-fibrotic signaling mechanisms, Ofev is intended to slow the progression of IPF.

Prilosec (omeprazole) is an oral proton-pump inhibitor that works by decreasing the amount of acid made in the stomach. It is used alone or with other medications to treat acid reflux, a condition in which acid from the stomach rises to the esophagus, causing heartburn and possible injury. Acid reflux is common in PF patients and may worsen other symptoms.

Tyvaso is an inhaled formulation of treprostinil approved for the treatment of pulmonary hypertension associated with interstitial lung disease (including IPF). It is a lab-made molecule that mimics the activity of prostacyclin, which works to widen blood vessels, so that blood flows more easily with less pressure. By helping blood vessels to relax and widen, Tyvaso is designed to improve blood circulation while keeping blood pressure low.