desert (a cause, a country or an army), often in order to join the opposing cause, country, or army; "If soldiers deserted Hitlers army, they were shot" (同)desert
an imperfection in an object or machine; "a flaw caused the crystal to shatter"; "if there are any defects you should send it back to the manufacturer" (同)fault, flaw
an imperfection in a bodily system; "visual defects"; "this device permits detection of defects in the lungs"
a failing or deficiency; "that interpretation is an unfortunate defect of our lack of information" (同)shortcoming
serving to restrict; "teenagers eager to escape restrictive home environments"
(of tariff) protective of national interests by restricting imports
a device (such as a fan) that introduces fresh air or expels foul air
provided with ventilation or involving pulmonary ventilation
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.
Contents
1Presentation
2Causes
2.1Intrinsic
2.2Extrinsic
3Pathophysiology
4Diagnosis
5Management
6See also
7References
8External links
Presentation
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
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
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
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
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
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
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
Chronic obstructive pulmonary disease
Extrapulmonary restriction
Obstructive lung disease
References
^Johns Hopkins School of Medicine's Interactive Respiratory Physiology > Restrictive Ventilatory Defect Retrieved on February 25, 2010
^ abCapriotti, Theresa (2016). Pathophysiology : introductory concepts and clinical perspectives. Frizzell, Joan Parker. Philadelphia. ISBN 978-0-8036-1571-7. OCLC 900626405.
^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.
^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.
^ abcFocus 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
Classification
D
ICD-9-CM: 518.89
External resources
eMedicine: article/301760
v
t
e
Diseases of the respiratory system
Upper RT (including URTIs, common cold)
Head
sinuses
Sinusitis
nose
Rhinitis
Vasomotor rhinitis
Atrophic rhinitis
Hay fever
Nasal polyp
Rhinorrhea
nasal septum
Nasal septum deviation
Nasal septum perforation
Nasal septal hematoma
tonsil
Tonsillitis
Adenoid hypertrophy
Peritonsillar abscess
Neck
pharynx
Pharyngitis
Strep throat
Laryngopharyngeal reflux (LPR)
Retropharyngeal abscess
larynx
Croup
Laryngomalacia
Laryngeal cyst
Laryngitis
Laryngopharyngeal reflux (LPR)
Laryngospasm
vocal cords
Laryngopharyngeal reflux (LPR)
Vocal fold nodule
Vocal fold paresis
Vocal cord dysfunction
epiglottis
Epiglottitis
trachea
Tracheitis
Laryngotracheal stenosis
Lower RT/lung disease (including LRTIs)
Bronchial/ obstructive
acute
Acute bronchitis
chronic
COPD
Chronic bronchitis
Acute exacerbation of COPD)
Asthma (Status asthmaticus
Aspirin-induced
Exercise-induced
Bronchiectasis
Cystic fibrosis
unspecified
Bronchitis
Bronchiolitis
Bronchiolitis obliterans
Diffuse panbronchiolitis
Interstitial/ restrictive (fibrosis)
External agents/ occupational lung disease
Pneumoconiosis
Aluminosis
Asbestosis
Baritosis
Bauxite fibrosis
Berylliosis
Caplan's syndrome
Chalicosis
Coalworker's pneumoconiosis
Siderosis
Silicosis
Talcosis
Byssinosis
Hypersensitivity pneumonitis
Bagassosis
Bird fancier's lung
Farmer's lung
Lycoperdonosis
Other
ARDS
Combined pulmonary fibrosis and emphysema
Pulmonary edema
Löffler's syndrome/Eosinophilic pneumonia
Respiratory hypersensitivity
Allergic bronchopulmonary aspergillosis
Hamman-Rich syndrome
Idiopathic pulmonary fibrosis
Sarcoidosis
Vaping-associated pulmonary injury
Obstructive / Restrictive
Pneumonia/ pneumonitis
By pathogen
Viral
Bacterial
Pneumococcal
Klebsiella
Atypical bacterial
Mycoplasma
Legionnaires' disease
Chlamydiae
Fungal
Pneumocystis
Parasitic
noninfectious
Chemical/Mendelson's syndrome
Aspiration/Lipid
By vector/route
Community-acquired
Healthcare-associated
Hospital-acquired
By distribution
Broncho-
Lobar
IIP
UIP
DIP
BOOP-COP
NSIP
RB
Other
Atelectasis
circulatory
Pulmonary hypertension
Pulmonary embolism
Lung abscess
Pleural cavity/ mediastinum
Pleural disease
Pleuritis/pleurisy
Pneumothorax/Hemopneumothorax
Pleural effusion
Hemothorax
Hydrothorax
Chylothorax
Empyema/pyothorax
Malignant
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Mediastinal disease
Mediastinitis
Mediastinal emphysema
Other/general
Respiratory failure
Influenza
Common cold
SARS
COVID-19
Idiopathic pulmonary haemosiderosis
Pulmonary alveolar proteinosis
UpToDate Contents
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…may identify air trapping or hyperinflation caused by airflow limitation. A concomitant restrictive ventilatory defect is detected in less than 10 percent of patients with a reduced FVC . Once the diagnosis …
…clinical examination may reveal fine inspiratory crackles and tachypnea . A mild-to-moderate restrictive ventilatory defect is most common, although lung function is occasionally normal. The DLCO is reduced in …
… while mild obstruction can be due to edema of small airways. Among patients with a restrictive ventilatory defect, a normal DLCO suggests an extrapulmonary cause of the restriction, such as pleural effusion …
… wall defect reduced alveolar oxygen levels in this area, thereby depriving the tuberculosis bacteremia of oxygen). Pulmonary function – Thoracoplasty results in a restrictive ventilatory defect with decreases …
…response to therapy, and prognosis . In patients with NSIP, PFTs typically demonstrate a restrictive ventilatory defect (decreased FVC and total lung capacity [TLC]) and impaired gas transfer manifested by …
A case of sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance : long-term observation of neurological symptoms after autologous stem-cell transplantation
A case of type 1 facioscapulohumeral muscular dystrophy (FSHD) with restrictiveventilatorydefect and congestive heart failure
Morimoto Nobutoshi,Morimoto Mizuki,Takahashi Yoshiaki,Takamiya Motonori,Nishino Ichizo,Abe Koji
eNeurologicalSci (21), 100284, 2020-12
… It was recently reported that restrictive respiratory involvement is more frequent and severe than previously recognized, while cardiac dysfunction other than arrhythmia is still considered extremely rare in FSHD. … A pulmonary function test revealed a decline of forced vital capacity (FVC) and a preserved FEV1/FVC indicating restrictiveventilatorydefect (RVD). …
Prevalence of Spirometrically-defined Restrictive Ventilatory Defect in Korea: The Fourth-2, 3, and Fifth Korean National Health and Nutrition Examination Survey, 2008-2012. Lee JY, Hwang YI, Park YB, Park JY, Kim KU, Oh YM, Yoon HK, Yoon HI, Sheen SS, Lee SY, Lee CH, Lee HB, Lim SC, Jung SS, Oh K, Kim Y, Chun C, Yoo KH J Korean Med Sci 2015 Jun;30(6):725-32.
A restrictive ventilatory defect in spirometry is a common finding (12.7%) with a highly variable geographical distribution (range 3.7) whose population burden is important in terms of quality of life and activities of daily living and similar to that of an obstructive pattern compatible with COPD.
A restrictive ventilatory defect is a common finding when conducting spirometry, with a prevalence in the general adult population ranging from 7% to 13% in a number of surveys. 1–3 The clinical ...