Interstitial (Nonidiopathic) Pulmonary Fibrosis Medication

Updated: Oct 16, 2018
  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Medication

Medication Summary

Medications are best used for specific diagnoses. However, corticosteroids, cytotoxic agents, and, more recently, antifibrotics, antioxidants, and other immunosuppressive agents have been used with varying success in some forms of DPLD. [24]

In general, NSIP, DIP, and COP have been found to be more responsive to corticosteroids and immunosuppressive therapies. UIP is generally thought to be unresponsive to these modalities, and thus, additional research in the form of clinical trials evaluating potentially promising agents continues. RBILD responds to smoking cessation.

Immunosuppressive and antifibrotic medications and supplemental oxygen may be indicated for some patients.

Prompt treatment is necessary for complicating pulmonary disease such as cor pulmonale (oxygen, diuretics), pulmonary embolism (anticoagulants), and infection (antibiotics).

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

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, corticosteroids modify body's immune response to diverse stimuli.

Prednisone (Rayos, Deltasone)

Used as immunosuppressant in treatment of autoimmune disorders. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation. Oral corticosteroid with relatively less mineralocorticoid activity.

Best prescribed in consultation with a pulmonary disease specialist.

Prednisolone (FloPred, Millipred, Millipred DP, Prelone)

Elicits mild mineralocorticoid activity and moderate anti-inflammatory effects; controls or prevents inflammation by controlling rate of protein synthesis, suppressing migration of polymorphonuclear leukocytes (PMNs) and fibroblasts, reversing capillary permeability, and stabilizing lysosomes at cellular level

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Immunosuppressants

Class Summary

These agents may inhibit key factors in involved in immune reactions.

Cyclophosphamide

Chemically related to nitrogen mustards. As an alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells of immune system. Possibly a steroid-sparing medication.

Azathioprine (Imuran, Azasan)

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

Cyclosporine (Gengraf, Neoral, Sandimmune)

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions.

Methotrexate (Otrexup, Rasuvo, Rheumatrex, Trexall)

Used for managing constitutional symptoms. It blocks purine synthesis and 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR), thus increasing anti-inflammatory adenosine concentration at sites of inflammation. Methotrexate ameliorates symptoms of inflammation.

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Anti-inflammatories

Class Summary

Immunosuppressive effects may inhibit cellular division and fibrosis.

Colchicine (Colcrys, Mitigare)

Decreases leukocyte motility and phagocytosis observed in inflammatory responses.

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Pulmonary, Tyrosine Kinase Inhibitors

Class Summary

Inhibition of various tyrosine kinases decreases the proliferative activities that lead to fibrosis.

Nintedanib (Ofev)

Nintedanib inhibits multiple tyrosine kinases and targets growth factors, which have been shown to be potentially involved in pulmonary fibrosis (eg, vascular endothelial growth factor receptor [VEGFR], fibroblast growth factor receptor [FGFR], platelet-derived growth factor receptor [PDGF]. It binds competitively to the adenosine triphosphate (ATP)-binding pocket of these receptors and blocks the intracellular signaling, which is crucial for the proliferation, migration, and transformation of fibroblasts, representing essential mechanisms of the idiopathic pulmonary fibrosis pathology.

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Transforming Growth Factor Inhibitors

Class Summary

Reduction of fibroblast proliferation may decrease the formation and/or accumulation of fibrotic materials within the lungs.

Pirfenidone (Esbriet)

The precise mechanism by which pirfenidone may work in pulmonary fibrosis has not been established. It inhibits transforming growth factor (TGF)-beta, a chemical mediator that controls many cell functions including proliferation and differentiation. It also inhibits the synthesis of TNF-alpha, a cytokine that is known to have an active role in inflammation.

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