The treatment of ILD depends on the degree of inflammation and scarring. Inflammation may be reversible or controlled with medical therapies such as steroids (like prednisone) or stronger medications, called immunosuppressive agents (such as azathioprine, mycophenelate mofetil, or cytoxan). The decision to use steroids or immunosuppressive medications depends on each patient's unique situation. An ILD specialist will discuss the risks and benefits of potential treatments with each patient.
For some patients, inflammation is present at very low levels or not at all, and scarring (fibrosis) predominates. In these cases, steroids or immunosuppressive therapy are unlikely to be beneficial because lung scarring is usually irreversible.
Some antifibrotic agents have been studied (such as pirfenidone) or are being studied (like N-acetylcysteine) for the treatment of idiopathic pulmonary fibrosis. The FDA has approved two antifibrotic agents (pirfenidone, nintedanib) for use in the treatment of patients with idiopathic pulmonary fibrosis. Our team can assist in ordering these medications as well as doing the monitoring as required. Clinical trials of investigational agents and/or referral for lung transplantation may be treatment options for those with progressive fibrosis.
Supportive therapies for ILD include the use of supplemental oxygen, pulmonary rehabilitation, treatment of coexisting pulmonary conditions (such as chronic obstructive pulmonary disease or obstructive sleep apnea) and or complications of ILD (such as pulmonary hypertension).