Restrictive Lung Disease Medication

Updated: Sep 16, 2020
  • Author: Jonathan Robert Caronia, DO; Chief Editor: John J Oppenheimer, MD  more...
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Medication

Medication Summary

Medications are best used for specific diagnoses. Corticosteroids, cytotoxic agents, and immunosuppressive agents have been used with varying success.

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and can modify the body's immune response.

Prednisone (Sterapred)

Prednisone is used as an immunosuppressant in the treatment of autoimmune disorders. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation. It is an oral corticosteroid with relatively less mineralocorticoid activity. Therapy is best prescribed in consultation with a pulmonary disease specialist.

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Cytotoxic agents

Class Summary

These agents inhibit cell growth and proliferation.

Cyclophosphamide (Cytoxan, Neosar)

Cyclophosphamide is chemically related to nitrogen mustards. As an alkylating agent, its mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with the growth of normal and neoplastic cells of the immune system. It is a possible steroid-sparing medication.

Azathioprine (Imuran)

Azathioprine inhibits mitosis and cellular metabolism by antagonizing purine metabolism and inhibiting the synthesis of DNA, RNA, and proteins. These effects may decrease the proliferation of immune cells and result in lower autoimmune activity. It is a possible steroid-sparing medication.

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Anti-inflammatory agents

Class Summary

These agents reduce inflammation by inhibiting key steps of the immune system.

Colchicine

Colchicine decreases leukocyte motility and phagocytosis observed in inflammatory responses.

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Pulmonary, Tyrosine Kinase Inhibitors

Class Summary

These agents target numerous intracellular pathways to reduce the fibrosis process by interrupting cell signaling pathways.

Nintedanib (Ofev)

Nintedanib is a tyrosine kinase inhibitor that targets growth factors that have been shown to potentially be involved in pulmonary fibrosis (eg, vascular endothelial growth factor receptor [VEGFR], fibroblast growth factor receptor [FGFR], platelet derived growth factor receptor [PDGF]).

Pirfenidone (Esbriet)

Pirfenidone is an inhibitor of transforming growth factor-beta (TGF-β), and it also inhibits the synthesis of tumor necrosis factor-alpha (TNF-α).

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