Restrictive Lung Disease Follow-up

  • Author: Lalit K Kanaparthi, MD; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: Jan 18, 2012
 

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.

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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.

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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.[25] IPF is typically a relentless progressive disorder, and patients have a mean survival of 3-6 years after diagnosis.[26] Early recognition of IPF is important for directing patient management and predicting prognosis.[27]
  • 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.
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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 Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

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.

Specialty Editor Board

Laurie Robin Grier, MD  Medical Director of MICU, Professor of Medicine, Department of Emergency Medicine, Anesthesiology and OBGYN, 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.

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: Nothing to disclose.

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.

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Approximately half of the patients with idiopathic pulmonary fibrosis develop clubbing. Clubbing is commonly seen in patients with asbestosis.
Lung volume is plotted against transpulmonary pressure. Compliance is the change in volume for a given change in pressure. A patient with emphysema has much higher lung compliance compared to a patient with intrinsic lung disease.
Idealized flow volume curves for normal, obstructive, and restrictive lungs.
The expiratory flow volume curves of 2 patients are depicted graphically. A is a patient with restrictive lung disease (idiopathic pulmonary fibrosis), low forced vital capacity (FVC), but an increased ratio of forced expiratory volume in 1 second (FEV1) to FVC because of increased elastic recoil. B is a patient with chronic obstructive lung disease whose FEV1/FVC ratio is low but whose lung volumes are increased.
Pulmonary function test results from a patient with restrictive lung disease.
Gross pathology of small and firm lungs due to restrictive lung disease from advanced pulmonary fibrosis.
Intrinsic lung disease may progress to extensive fibrosis, regardless of etiology. This is described as honeycomb lung.
End-stage sarcoidosis.
Usual interstitial pneumonitis (left).
Usual interstitial pneumonitis (right).
Histopathology of a case of idiopathic pulmonary fibrosis. Alveolitis with fibroblast proliferation and collagen deposition is present.
In usual interstitial pneumonitis or idiopathic pulmonary fibrosis, subpleural and paraseptal inflammation is present, with an appearance of temporal heterogeneity. Patchy scarring of the lung parenchyma and normal, or nearly normal, alveoli interspersed between fibrotic areas are the hallmarks of this disease. Additionally, the lung architecture is completely destroyed.
Characteristic features of usual interstitial pneumonitis as described in the image below.
Bronchiolitis obliterans-organizing pneumonia (also called proliferative bronchiolitis) is often patchy and peribronchiolar. The proliferation of granulation tissue within small airways and alveolar ducts is excessive and is associated with chronic inflammation of surrounding alveoli.
Bronchiolitis obliterans-organizing pneumonia, as described in the image below, showing a close-up view of fibrogranulation tissue in terminal airspaces.
Granulomatous lung diseases are marked by granulomas characterized by the accumulation of T lymphocytes, macrophages, and epithelioid cells. These may progress to pulmonary fibrosis. This low-power image shows well-formed granuloma along the airway.
Multiple well-formed noncaseating granulomas secondary to sarcoidosis.
Sarcoid granulomas.
High-power view of sarcoid granuloma shows giant cells.
A patient who developed restrictive lung disease had findings of bronchiolitis obliterans-organizing pneumonia on an open lung biopsy specimen.
A patient who developed restrictive lung disease had findings of bronchiolitis obliterans-organizing pneumonia on an open lung biopsy specimen. The biopsy sample shows intraluminal buds of granulation tissue.
Lymphocytic interstitial pneumonitis, for which the prominent finding is a lymphoid infiltrate that involves both the interstitium and alveolar spaces.
Usual interstitial pneumonitis honeycombing.
Chest radiograph of a 67-year-old man diagnosed with idiopathic pulmonary fibrosis, based on open lung biopsy findings. Extensive bilateral reticulonodular opacities are seen in both lower lobes.
High-resolution CT scan of the same patient in the image below demonstrates peripheral honeycombing and several areas of ground-glass attenuation. Ground-glass opacification may correlate with active alveolitis and a favorable response to therapy.
A CT scan image from a 59-year-old woman shows advanced pulmonary fibrosis. Extensive honeycombing and traction bronchiectasis are present.
Restrictive lung disease may occur in stage II and stage III sarcoidosis. In this image, mediastinal lymphadenopathy is shown secondary to stage II disease.
Sarcoidosis on CT scan shows nodules in midlung zones. These nodules are predominantly along the bronchovascular bundles and in a subpleural location.
Restrictive lung disease secondary to sarcoidosis.
A chest radiograph of stage III sarcoidosis. This stage refers to pulmonary infiltrates without evidence of mediastinal lymphadenopathy.
Chest radiograph from a 39-year-old woman with severe kyphoscoliosis who developed hypercapnic respiratory failure. Spirometry findings showed a severe restrictive lung disease, with a forced expiratory volume in one second of 0.4 L/s and a forced vital capacity of 0.5 L.
The flow volume curve of a patient with lung fibrosis.
Likely case of idiopathic pulmonary fibrosis, which should be treated with prednisone.
Table. Contrasting Clinical, Radiologic, and Histologic Features of Acute Interstitial Pneumonia (AIP), Usual Interstitial Pneumonia (UIP), Nonspecific Interstitial Pneumonia (NSIP),[14] and BOOP[15]
FeaturesAIPUIPNSIPBOOP
Pathologic
Temporal appearanceUniformHeterogeneousUniformUniform
Interstitial inflammationScantScantUsually prominentVariable
Collagen fibrosisNoPatchyVariable, diffuseNo
Fibroblast proliferationDiffuse, interstitialPatchy (fibroblast foci)OccasionalPatchy, airspace
BOOP areasRareNoRare--
Honeycomb changesRareYesRareNo
Hyaline membranesYes, often focalNoNoNo
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