Interstitial (Nonidiopathic) Pulmonary Fibrosis Treatment & Management

  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: Jun 13, 2011
 

Medical Care

  • Treatment is best determined by the specific diagnosis. Unfortunately, a specific etiology often is not determined. General supportive measures include the following:
    • Smoking cessation should be counseled.
    • Good pulmonary hygiene is important.
    • Supplemental oxygen therapy may be useful for any patients who demonstrate significant hypoxemia (oxygen saturation [SaO2] less than or equal to 88% or PaO2 < 55 mm Hg while breathing room air either at rest or upon exertion).
    • If inhalational exposures are thought to be the etiology, as in HP and occupational forms of interstitial lung disease, removal from exposure is mandatory.
    • If a toxic medication is suspected, its discontinuation is mandated.
    • Respiratory infections should be promptly treated.
    • Pulmonary rehabilitation may improve exercise tolerance and quality of life.
  • 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,[9] pirfenidone,[10] and N-acetylcysteine[11] 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.
  • 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.[12] 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.[13]
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Surgical Care

  • Surgery in the form of either thoracoscopic (preferred) or open lung biopsy is often indicated to obtain tissue specimens for definitive diagnosis.
  • More recently, lung transplantation has become a treatment option for selected patients with advanced disease refractory to medical therapy.[14] It is the only interventional modality that has been shown to increase survival time in patients with UIP/IPF.[15, 16]
    • Survival rates worldwide after single lung transplantation are approximately 74% at 1 year, 58% at 3 years, 47% at 5 years, and 24% at 10 years.
    • Survival rates are lower for bilateral lung transplantation.
    • Following transplantation, patients overall report improved quality of life with better physical, social, and general health functioning.
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Consultations

  • Consider consultation with a pulmonary or occupational disease specialist for patients with suspected DPLD.
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Diet

  • No specific dietary restrictions are warranted for affected patients.
  • Some suggest that antioxidants have a therapeutic benefit.
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Activity

  • Encourage exercise and pulmonary rehabilitation because they may improve a patient's functional status. However, these activities generally have no effect on disease progression.
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Contributor Information and Disclosures
Author

Eleanor M Summerhill, MD, FACP, FCCP  Associate Professor of Medicine, Division of Pulmonary and Critical Care Medicine, Warren Alpert Medical School of Brown University; Director, Internal Medicine Residency Program, Memorial Hospital of Rhode Island

Eleanor M Summerhill, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Thoracic Society, Association of Program Directors in Internal Medicine, and Rhode Island Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP  Professor of Genomics and Personalized Medicine Research, Internal Medicine, and Pediatrics, Associate Director, Center for Genomics and Personalized Medicine Research, Director of Research, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Wake Forest University School of Medicine

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, and Sigma Xi

Disclosure: See below for list of all activities None None

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Daniel R Ouellette, MD, FCCP  Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society

Disclosure: Boehringer Ingleheim Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Astra Zeneca Honoraria Speaking and teaching

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Robert S. Crausman, MD, MMS, to the development and writing of this article.

References

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Frontal chest radiograph demonstrating bilateral reticular and nodular interstitial infiltrates with upper zone predominance.
High-resolution chest CT scan of patient with bilateral reticular and nodular interstitial infiltrates with upper zone predominance.
 
 
 
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