eMedicine Specialties > Pulmonology > Interstitial Lung Diseases

Restrictive Lung Disease: Follow-up

Author: Lalit K Kanaparthi, MD, Senior Fellow, Department of Pulmonary Medicine, Lenox Hill Hospital
Coauthor(s): Klaus-Dieter Lessnau, MD, FCCP, Clinical Associate Professor of Medicine, New York University School of Medicine; Medical Director, Pulmonary Physiology Laboratory; Director of Research in Pulmonary Medicine, Department of Medicine, Section of Pulmonary Medicine, Lenox Hill Hospital; Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital
Contributor Information and Disclosures

Updated: Jul 27, 2009

Follow-up

Deterrence/Prevention

Acute exacerbation in patients with idiopathic pulmonary fibrosis (IPF) is a recently recognized complication that occurs unpredictably and presents as worsening dyspnea. Chest radiography demonstrates bilateral mixed alveolar-interstitial infiltrates and CT scan reveals ground-glass opacities and consolidation. The treatment includes high-dose systemic corticosteroids, although these are likely not effective, and the disease portends extremely poor prognosis.

Complications

Acute exacerbation in patients with IPF is a recently recognized complication that occurs unpredictably and presents as worsening dyspnea. Chest radiography demonstrates bilateral mixed alveolar-interstitial infiltrates and CT scan reveals ground-glass opacities and consolidation. The treatment includes high-dose systemic corticosteroids, although these are likely not effective, and the disease portends extremely poor prognosis.

Prognosis

  • The natural history of interstitial lung diseases is variable. It depends on the specific diagnosis and the extent and severity of lung involvement based on high-resolution CT scanning and lung biopsy.21 IPF is typically a relentless progressive disorder, and patients have a mean survival of 3-6 years after diagnosis.22 Early recognition of IPF is important for directing patient management and predicting prognosis.23
  • Pulmonary sarcoidosis has a relatively benign self-limiting course, with spontaneous recovery or stabilization in most cases. Approximately 15% of patients develop pulmonary fibrosis and disability.
  • Prognosis for collagen-vascular diseases, eosinophilic pneumonia, BOOP, and drug-induced lung disease is generally favorable with treatment.
  • Patients with chest wall diseases and neuromuscular disorders develop progressive respiratory failure and succumb during an intercurrent pulmonary infection.

Miscellaneous

Medicolegal Pitfalls

  • Irrespective of lung biopsy findings, if patients are symptomatic, they should receive a trial period of therapy. For many years, the absolute standard for diagnosis of IPF was purported to be surgical lung biopsy. This theory (and subsequent biopsy findings) helped differentiate patients with cellular, as opposed to fibrotic, disease. In practice, the same histologic patterns are seen in both types of patients.
  • Therapeutic options for IPF are limited. Drugs with antifibrotic properties or anti-inflammatory agents that work against growth factors and suppress inflammation are needed.
  • An absolute requirement is that all patients with restrictive lung disease must be differentiated as having either intrinsic or extrinsic disorders, the determination of which is based on pulmonary function test findings.

Special Concerns

  • The clinical course of IPF is variable. In most cases, the course involves a progressive deterioration culminating in death from respiratory failure. The secular survival interval expectation among newly diagnosed patients is typically 3-5 years.
  • A low FVC, low DLCO, low arterial oxygen at presentation, male sex, and older age are markers of a poor prognosis.
  • Improvement after a trial corticosteroid therapy is associated with a favorable prognosis and is more probable in patients with cellular changes, which may be noted on lung biopsy findings, or ground-glass attenuation, which may be noted on a high-resolution CT scan image.
 


More on Restrictive Lung Disease

Overview: Restrictive Lung Disease
Differential Diagnoses & Workup: Restrictive Lung Disease
Treatment & Medication: Restrictive Lung Disease
Follow-up: Restrictive Lung Disease
Multimedia: Restrictive Lung Disease
References
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References

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Further Reading

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Keywords

restrictive lung disease, pulmonary fibrosis, kyphoscoliosis, sarcoidosis, interstitial pneumonitis, intrinsic lung diseases, pneumonitis, diseases of lung parenchyma, interstitial lung disease, pneumonitis, idiopathic fibrotic diseases, connective-tissue diseases, drug-induced lung disease, primary lung disease, extrinsic lung disorders, extraparenchymal diseases, lung restriction, impaired ventilatory function, respiratory failure, idiopathic pulmonary fibrosis, IPF, total lung capacity, TLC

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Contributor Information and Disclosures

Author

Lalit K Kanaparthi, MD, Senior Fellow, Department of Pulmonary Medicine, Lenox Hill Hospital
Lalit K Kanaparthi, MD is a member of the following medical societies: American College of Chest Physicians, American Medical Association, and American Thoracic Society
Disclosure: Nothing to disclose.

Coauthor(s)

Klaus-Dieter Lessnau, MD, FCCP, Clinical Associate Professor of Medicine, New York University School of Medicine; Medical Director, Pulmonary Physiology Laboratory; Director of Research in Pulmonary Medicine, Department of Medicine, Section of Pulmonary Medicine, Lenox Hill Hospital
Klaus-Dieter Lessnau, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Society for Artificial Internal Organs, American Thoracic Society, Physicians for Social Responsibility, and Society of Critical Care Medicine
Disclosure: sepracor Ownership interest None

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Laurie Robin Grier, MD, Medical Director of MICU, Associate Professor of Medicine, Section of Pulmonary and Critical Care Medicine, Louisiana State University Health Science Center at Shreveport
Laurie Robin Grier, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Society for Parenteral and Enteral Nutrition, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint 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, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
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.

 
 
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