Fast Five Quiz: Interstitial Lung Disease Progressive Fibrosing Management

Zab Mosenifar, MD


March 06, 2023

Patients with PPF due to autoimmune conditions, such as rheumatoid arthritis and systemic sclerosis, have generally been treated with immunosuppressive therapy depending on the underlying ILD subtype. Immunosuppressant agents such as corticosteroids, mycophenolate mofetil (MMF), azathioprine, methotrexate, and cyclophosphamide may target the inflammatory components of PPF.

MMF is the most widely used first-line agent in fibrosing ILDs as it is generally effective and well tolerated. MMF has been shown to improve or stabilize FVC and DLCO in patients with PPF associated with connective tissue disorders. Similarly, a recent study suggests that combination therapy with prednisone and MMF may decrease disease progression.

Short-term corticosteroid therapy may help to stabilize rapidly progressive disease; however, corticosteroids are generally not used long-term, if possible, due to their side effects. Corticosteroids are frequently used in the treatment of other non-autoimmune PPFs such as hypersensitivity pneumonitis.

Learn more about medication for PF-ILD.


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