What is the role of pharmacologic therapy in the treatment of diffuse parenchymal lung diseases (DPLDs)?

Updated: Sep 15, 2020
  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Pharmacologic therapy with corticosteroids (eg, prednisone) and/or cytotoxic agents for their potential steroid-sparing effect (eg, cyclophosphamide, azathioprine, methotrexate) may be indicated for specific diagnoses.

Other immunosuppressive or antifibrotic agents such as colchicine, cyclosporine, and D-penicillamine may have a role in specific cases. Empiric use of these medications without a specific diagnosis should be discouraged because they have significant toxicities.

Interferon-gamma-1b, [12] pirfenidone, [13] and N-acetylcysteine [14] have been studied for the treatment of IPF. Interferon-gamma-1b initially appeared to have a favorable effect. This, however, was not supported in a larger follow-up study. Some evidence suggests that pirfenidone and acetylcysteine may have some benefit in IPF. Further investigation is still needed, particularly in other forms of DPLD. As much remains unknown regarding the optimal therapy for DPLD, eligible patients may benefit from enrollment in an experimental trial.

Pirfenidone and nintedanib are approved by the US Food and Drug Administration for IPF treatment. [15, 16, 17]

A 2008 multisystem, randomized, controlled study of bosentan, an endothelin-1 receptor antagonist, and potentially anti-fibrotic agent, did not show superiority over placebo. However, a trend toward delay in progression of disease and improvement in mortality was noted, which was more pronounced in patients with UIP documented by surgical biopsy. [18] Additional phase III trials are ongoing.

Other novel potential therapeutic agents, such as recombinant TNF-alpha antagonists and tyrosine kinase inhibitors, are currently under investigation. These agents are further described in a recently published comprehensive review. [19]

Most patients with DPLD can be treated in community settings. Transfer to a tertiary care center is indicated when the diagnosis is in doubt or when treatment is ineffective.

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