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Restrictive lung disease

January 01, 1970

Restrictive lung diseases 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.

Restrictive lung disease
Other namesRestrictive ventilatory defect[1]
SpecialtyPulmonology

Presentation edit

Due to the chronic nature of this disease, the leading symptom of restrictive lung disease is progressive exertional dyspnea.[3]  For acute on chronic cases, shortness of breath, cough, and respiratory failure are some of the more common signs.[3]

Causes edit

Restrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung, or extrinsic to it.[3]

Intrinsic edit

Many cases of restrictive lung disease are idiopathic (have no known cause). Still, there is generally pulmonary fibrosis.[5] Examples are:

Conditions specifically affecting the interstitium are called interstitial lung diseases.

Extrinsic edit

Pathophysiology edit

In normal respiratory function, the air flows in through the upper airway, down through the bronchi and into the lung parenchyma (the bronchioles down to the alveoli) where gas exchange of carbon dioxide and oxygen occurs.[7] During inspiration, the lungs expand to allow airflow into the lungs and thereby increasing total volume. After inspiration follows expiration during which the lungs recoil and push air back out of the pulmonary pathway. Lung compliance is the difference of volume during inspiration and expiration.[7]

Restrictive lung disease is characterized by reduced lung volumes, and therefore reduced lung compliance, either due to an intrinsic reason, for example a change in the lung parenchyma, or due to an extrinsic reason, for example diseases of the chest wall, pleura, or respiratory muscles.[3] Generally, intrinsic causes are from lung parenchyma diseases that cause inflammation or scarring of the lung tissue, such as interstitial lung disease or pulmonary fibrosis, or from having the alveoli air spaces filled with external material such as debris or exudate in pneumonitis.[3] As some diseases of the lung parenchyma progress, the normal lung tissue can be gradually replaced with scar tissue that is interspersed with pockets of air.[5] This can lead to parts of the lung having a honeycomb-like appearance. The extrinsic causes result in lung restriction, impaired ventilatory function, and even respiratory failure due to the diseases that effect the lungs ability to create a change in lung volumes during respiration due to the diseases of the systems stated above.[3]

Diagnosis edit

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 0.7, indicating that FEV1 is significantly reduced when compared to the total expired volume. This indicates that the FVC is also reduced, but not by the same ratio as FEV1.[8]

One definition requires a total lung capacity which is 80% or less of the expected value.[9]

Management edit

Medical treatment for restrictive lung disease is normally limited to supportive care since both the intrinsic and extrinsic causes can have irreversible effects on lung compliance.[10] The supportive therapies focus on maximizing pulmonary function and preserving activity tolerance through oxygen therapy, bronchodilators, inhaled beta-adrenergic agonists, and diuretics.[10] Because there is no effective treatment for restrictive lung disease, prevention is key.[10]

See also edit

References edit

  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 c d e f "eMedicine - Restrictive Lung Disease : Article by Sat Sharma". Retrieved 2008-11-21.
  4. ^ Amaral, André F.S.; Coton, Sonia; Kato, Bernet; Tan, Wan C.; Studnicka, Michael; Janson, Christer; Gislason, Thorarinn; Mannino, David; Bateman, Eric D.; Buist, Sonia; Burney, Peter G.J. (October 2015). "Tuberculosis associates with both airflow obstruction and low lung function: BOLD results". European Respiratory Journal. 46 (4): 1104–1112. doi:10.1183/13993003.02325-2014. PMC 4594762. PMID 26113680.
  5. ^ a b PULMONARY FUNCTION TESTS A Workshop on Simple Spirometry & Flow Volume Loops. 2010-03-31 at the Wayback Machine Dr. S. Osborne, Dept. Cellular & Physiological Sciences. Mars 2009
  6. ^ 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
  7. ^ a b Capriotti, Theresa (2016). Pathophysiology : introductory concepts and clinical perspectives. Frizzell, Joan Parker. Philadelphia. ISBN 978-0-8036-1571-7. OCLC 900626405.{{cite book}}: CS1 maint: location missing publisher (link)
  8. ^ 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, Vol.191.
  9. ^ 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. doi:10.1164/rccm.2201018. PMID 11991875.
  10. ^ a b c Focus on adult health : medical-surgical nursing. Honan, Linda, 1955- (1st ed.). Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins. 2013. ISBN 978-1-58255-877-6. OCLC 756895022.{{cite book}}: CS1 maint: others (link)

External links edit

January 01, 1970
restrictive, lung, disease, category, extrapulmonary, pleural, parenchymal, respiratory, diseases, that, restrict, lung, expansion, resulting, decreased, lung, volume, increased, work, breathing, inadequate, ventilation, oxygenation, pulmonary, function, test,. Restrictive lung diseases 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 Restrictive lung diseaseOther namesRestrictive ventilatory defect 1 SpecialtyPulmonology Contents 1 Presentation 2 Causes 2 1 Intrinsic 2 2 Extrinsic 3 Pathophysiology 4 Diagnosis 5 Management 6 See also 7 References 8 External linksPresentation editDue to the chronic nature of this disease the leading symptom of restrictive lung disease is progressive exertional dyspnea 3 For acute on chronic cases shortness of breath cough and respiratory failure are some of the more common signs 3 Causes editRestrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung or extrinsic to it 3 Intrinsic edit 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 Tuberculosis 4 Many cases of restrictive lung disease are idiopathic have no known cause Still there is generally pulmonary fibrosis 5 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 Extrinsic edit Nonmuscular diseases of the upper thorax such as kyphosis pectus carinatum and pectus excavatum 6 Diseases restricting lower thoracic abdominal volume e g obesity diaphragmatic hernia or the presence of ascites 6 Pleural thickening Pathophysiology editIn normal respiratory function the air flows in through the upper airway down through the bronchi and into the lung parenchyma the bronchioles down to the alveoli where gas exchange of carbon dioxide and oxygen occurs 7 During inspiration the lungs expand to allow airflow into the lungs and thereby increasing total volume After inspiration follows expiration during which the lungs recoil and push air back out of the pulmonary pathway Lung compliance is the difference of volume during inspiration and expiration 7 Restrictive lung disease is characterized by reduced lung volumes and therefore reduced lung compliance either due to an intrinsic reason for example a change in the lung parenchyma or due to an extrinsic reason for example diseases of the chest wall pleura or respiratory muscles 3 Generally intrinsic causes are from lung parenchyma diseases that cause inflammation or scarring of the lung tissue such as interstitial lung disease or pulmonary fibrosis or from having the alveoli air spaces filled with external material such as debris or exudate in pneumonitis 3 As some diseases of the lung parenchyma progress the normal lung tissue can be gradually replaced with scar tissue that is interspersed with pockets of air 5 This can lead to parts of the lung having a honeycomb like appearance The extrinsic causes result in lung restriction impaired ventilatory function and even respiratory failure due to the diseases that effect the lungs ability to create a change in lung volumes during respiration due to the diseases of the systems stated above 3 Diagnosis editIn 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 0 7 indicating that FEV1 is significantly reduced when compared to the total expired volume This indicates that the FVC is also reduced but not by the same ratio as FEV1 8 One definition requires a total lung capacity which is 80 or less of the expected value 9 Management editMedical treatment for restrictive lung disease is normally limited to supportive care since both the intrinsic and extrinsic causes can have irreversible effects on lung compliance 10 The supportive therapies focus on maximizing pulmonary function and preserving activity tolerance through oxygen therapy bronchodilators inhaled beta adrenergic agonists and diuretics 10 Because there is no effective treatment for restrictive lung disease prevention is key 10 See also editChronic obstructive pulmonary disease Extrapulmonary restriction Obstructive lung diseaseReferences edit Johns Hopkins School of Medicine s Interactive Respiratory Physiology gt Restrictive Ventilatory Defect Retrieved on February 25 2010 Sharma Sat Restrictive Lung Disease Retrieved 2008 04 19 a b c d e f eMedicine Restrictive Lung Disease Article by Sat Sharma Retrieved 2008 11 21 Amaral Andre F S Coton Sonia Kato Bernet Tan Wan C Studnicka Michael Janson Christer Gislason Thorarinn Mannino David Bateman Eric D Buist Sonia Burney Peter G J October 2015 Tuberculosis associates with both airflow obstruction and low lung function BOLD results European Respiratory Journal 46 4 1104 1112 doi 10 1183 13993003 02325 2014 PMC 4594762 PMID 26113680 a b PULMONARY FUNCTION TESTS A Workshop on Simple Spirometry amp Flow Volume Loops Archived 2010 03 31 at the Wayback Machine Dr S Osborne Dept Cellular amp Physiological Sciences Mars 2009 a b eMedicine Specialties gt Pulmonology gt Interstitial Lung Diseases gt Restrictive Lung Disease Author Lalit K Kanaparthi MD Klaus Dieter Lessnau MD Sat Sharma MD Updated Jul 27 2009 a b Capriotti Theresa 2016 Pathophysiology introductory concepts and clinical perspectives Frizzell Joan Parker Philadelphia ISBN 978 0 8036 1571 7 OCLC 900626405 a href Template Cite book html title Template Cite book cite book a CS1 maint location missing publisher link Lee H Lim S Kim J Ha H amp 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 Vol 191 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 doi 10 1164 rccm 2201018 PMID 11991875 a b c Focus on adult health medical surgical nursing Honan Linda 1955 1st ed Philadelphia Wolters Kluwer Health Lippincott Williams amp Wilkins 2013 ISBN 978 1 58255 877 6 OCLC 756895022 a href Template Cite book html title Template Cite book cite book a CS1 maint others link External links edit Retrieved from https en wikipedia org w index php title Restrictive lung disease amp oldid 1179866950, wikipedia, wiki, book, books, library,

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