Restrictive lung disease


Restrictive lung diseases or restrictive ventilatory defects1 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

  • 1 Pathophysiology
  • 2 Presentation
  • 3 Diagnosis
  • 4 Causes and classification
    • 41 Intrinsic
    • 42 Extrinsic
  • 5 See also
  • 6 References

Pathophysiologyedit

In disorders that are intrinsic to the lung parenchyma, the underlying process is usually pulmonary fibrosis scarring of the lung3 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

Presentationedit

The main symptoms are shortness of breath and coughcitation needed

Diagnosisedit

In restrictive lung disease, both forced expiratory volume in one second FEV1 and forced vital capacity FVC are reduced, however, the decline in FVC is more than that of FEV1, resulting in a higher than 80% FEV1/FVC ratio In obstructive lung disease however, the FEV1/FVC is less than 07, indicating that both FEV1 and FVC are reduced This indicates that the FVC is also reduced, but not by the same ratio as FEV14

One definition requires a total lung capacity which is 80% or less of the expected value5

Causes and classificationedit

Restrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung, or extrinsic to it6

Intrinsicedit

  • Pneumoconiosis caused by long-term exposure to dusts, especially in mining For example, Asbestosis
  • Radiation fibrosis, usually from the radiation given for cancer treatment
  • Certain drugs such as amiodarone, bleomycin and methotrexate
  • As a consequence of another disease such as rheumatoid arthritis
  • Hypersensitivity pneumonitis due to an allergic reaction to inhaled particles
  • Acute respiratory distress syndrome ARDS, a severe lung condition occurring in response to a critical illness or injury
  • Infant respiratory distress syndrome due to a deficiency of surfactant in the lungs of a baby born prematurely
  • Tuberculosis7

Many cases of restrictive lung disease are idiopathic have no known cause Still, there is generally pulmonary fibrosis3 Examples are:

  • Idiopathic pulmonary fibrosis
  • Idiopathic interstitial pneumonia, of which there are several types
  • Sarcoidosis
  • Eosinophilic pneumonia
  • Lymphangioleiomyomatosis
  • Pulmonary Langerhans' cell histiocytosis
  • Pulmonary alveolar proteinosis

Conditions specifically affecting the interstitium are called interstitial lung diseases

Extrinsicedit

  • Nonmuscular diseases of the upper thorax such as kyphosis, pectus carinatum and pectus excavatum8
  • Diseases restricting lower thoracic/abdominal volume eg obesity, diaphragmatic hernia, or the presence of ascites8
  • Pleural thickening

See alsoedit

  • Chronic obstructive pulmonary disease
  • Obstructive lung disease

Referencesedit

  1. ^ Johns Hopkins School of Medicine's Interactive Respiratory Physiology > Restrictive Ventilatory Defect Retrieved on February 25, 2010
  2. ^ Sharma, Sat "Restrictive Lung Disease" 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. ^ Lee, H, Lim, S, Kim, J, Ha, H, & Park, H 2015 Comparison Of Various Pulmonary Function Parameters In The Diagnosis Of Obstructive Lung Disease In Patients With Normal Fev1/FVC And Low FVC American Journal Of Respiratory And Critical Care Medicine, 191, American Journal Of Respiratory And Critical Care Medicine, 2015, Vol191
  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 doi:101164/rccm2201018 
  6. ^ "eMedicine - Restrictive Lung Disease : Article by Sat Sharma" Retrieved 2008-11-21 
  7. ^ Amaral, André FS; Coton, Sonia; Kato, Bernet; Tan, Wan C; Studnicka, Michael; Janson, Christer; Gislason, Thorarinn; Mannino, David; Bateman, Eric D; Buist, Sonia; Burney, Peter GJ October 2015 "Tuberculosis associates with both airflow obstruction and low lung function: BOLD results" European Respiratory Journal 46 4: 1104–1112 PMC 4594762  PMID 26113680 doi:101183/1399300302325-2014 
  8. ^ a b 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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