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Restrictive lung disease
Classification and external resources
ICD-9 518.89
eMedicine med/2012

Restrictive lung diseases (or restrictive ventilatory defects[1])are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion[2], resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.

Contents

Pathophysiology

In disorders that are intrinsic to the lung parenchyma, the underlying process is usually pulmonary fibrosis (scarring of the lung).[3] As the disease progresses, the normal lung tissue is gradually replaced by scar tissue interspersed with pockets of air. This can lead to parts of the lung having a honeycomb-like appearance.

Presentation

The main symptoms are shortness of breath and cough.

Diagnosis

In restrictive lung disease, both the FEV1 and FVC are reduced so the FEV1/FVC ratio is normal or even increased in contrast to obstructive lung disease where this ratio is reduced. The values for residual volume and total lung capacity are generally decreased in restrictive lung disease[4].

One definition requires a total lung capacity which is 80% or less of the expected value.[5]

Causes and classification

Restrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung, or extrinsic to it.[6]

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Intrinsic

Many cases of restrictive lung disease are idiopathic (have no known cause). Still, there is generally pulmonary fibrosis.[3] Examples are:

Conditions specifically affecting the interstitium are called interstitial lung diseases.

Extrinsic

See Also

Chronic obstructive pulmonary disease

References

  1. ^ Johns Hopkins School of Medicine's Interactive Respiratory Physiology > Restrictive Ventilatory Defect Retrieved on February 25, 2010
  2. ^ Sharma, Sat. "Restrictive Lung Disease". http://www.emedicine.com/med/topic2012.htm. Retrieved 2008-04-19. 
  3. ^ a b PULMONARY FUNCTION TESTS A Workshop on Simple Spirometry & Flow Volume Loops. Dr. S. Osborne, Dept. Cellular & Physiological Sciences. Mars 2009
  4. ^ "www.spirXpert.com". http://www.spirxpert.com/characteristic6.htm. 
  5. ^ Brack T, Jubran A, Tobin MJ (May 2002). "Dyspnea and decreased variability of breathing in patients with restrictive lung disease". Am. J. Respir. Crit. Care Med. 165 (9): 1260–4. PMID 11991875. http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=11991875. 
  6. ^ "eMedicine - Restrictive Lung Disease : Article by Sat Sharma". http://www.emedicine.com/med/topic2012.htm. Retrieved 2008-11-21. 
  7. ^ Walker J, Cooney M, Norton S (August 1989). "Improved pulmonary function in chronic quadriplegics after pulmonary therapy and arm ergometry". Paraplegia 27 (4): 278–83. PMID 2780083. 
  8. ^ eMedicine Specialties > Pulmonology > Interstitial Lung Diseases > Restrictive Lung Disease Author: Lalit K Kanaparthi, MD, Klaus-Dieter Lessnau, MD, Sat Sharma, MD. Updated: Jul 27, 2009

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