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==Overview==
==Overview==
Restrictive lung disease is a group of diseases that present with a restrictive pattern (a decrease in total lung capacity (TLC), respiratory volume (RV), forced vital capacity (FVC), forced expiatory volume (FEV1), and a normal to increased FEV1/FVC) on pulmonary function tests. These diseases include [[acute respiratory distress syndrome]],
Restrictive lung disease is a group of diseases that present with a restrictive pattern (a decrease in total lung capacity (TLC), respiratory volume (RV), forced vital capacity (FVC), forced expiatory volume (FEV1), and a normal to increased FEV1/FVC) on spirometry. These diseases include [[acute respiratory distress syndrome]],
[[hypersensitivity pneumonitis]], [[occupational lung disease|occupational lung diseases]], [[pleural effusion]], [[interstitial lung disease]], [[sarcoidosis]], and neuromuscular diseases such as scoliosis, [[muscular dystrophy]], [[ALS|amyotropic lateral sclerosis (ALS)]], and [[myasthenia gravis]]Restrictive lung disease must be differentiated from other diseases that cause [[dyspnea]], [[cough]], [[hemoptysis]], and [[fever]] such as [[Acute respiratory distress syndrome|ARDS]], [[hypersensitivity pneumonitis]], [[pneumoconiosis]], [[sarcoidosis]], [[pleural effusion]], [[Interstitial lung disease|interstitial lung disease (ILD)]], [[lymphocytic interstitial pneumonia]], [[obesity]], [[pulmonary eosinophilia]] and [[Neuromuscular disorder|neuromuscular disorders]].
[[hypersensitivity pneumonitis]], [[occupational lung disease|occupational lung diseases]], [[pleural effusion]], [[interstitial lung disease]], [[sarcoidosis]], and neuromuscular diseases such as scoliosis, [[muscular dystrophy]], [[ALS|amyotropic lateral sclerosis (ALS)]], and [[myasthenia gravis]]Restrictive lung disease must be differentiated from other diseases that cause [[dyspnea]], [[cough]], [[hemoptysis]], and [[fever]] such as [[Acute respiratory distress syndrome|ARDS]], [[hypersensitivity pneumonitis]], [[pneumoconiosis]], [[sarcoidosis]], [[pleural effusion]], [[Interstitial lung disease|interstitial lung disease (ILD)]], [[lymphocytic interstitial pneumonia]], [[obesity]], [[pulmonary eosinophilia]] and [[Neuromuscular disorder|neuromuscular disorders]].



Revision as of 21:32, 7 March 2018

Restrictive Lung Disease Microchapters

Overview

Classification

Acute respiratory distress syndrome
Hypersensitivity pneumonitis
Occupational lung diseases
Pleural Effusion
Interstitial lung disease
Sarcoidosis
Neuromuscular diseases
Scoliosis
Muscular dystrophy
Amyotropic lateral sclerosis (ALS)
Myasthenia gravis

Spirometry Findings in Various Lung Conditions

Approach to Lung Disorders

Differentiating Restrictive Lung Disease from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2], Akshun Kalia M.B.B.S.[3], Usama Talib, BSc, MD [4], Cafer Zorkun, M.D., Ph.D. [5]

Overview

Restrictive lung disease is a group of diseases that present with a restrictive pattern (a decrease in total lung capacity (TLC), respiratory volume (RV), forced vital capacity (FVC), forced expiatory volume (FEV1), and a normal to increased FEV1/FVC) on spirometry. These diseases include acute respiratory distress syndrome, hypersensitivity pneumonitis, occupational lung diseases, pleural effusion, interstitial lung disease, sarcoidosis, and neuromuscular diseases such as scoliosis, muscular dystrophy, amyotropic lateral sclerosis (ALS), and myasthenia gravisRestrictive lung disease must be differentiated from other diseases that cause dyspnea, cough, hemoptysis, and fever such as ARDS, hypersensitivity pneumonitis, pneumoconiosis, sarcoidosis, pleural effusion, interstitial lung disease (ILD), lymphocytic interstitial pneumonia, obesity, pulmonary eosinophilia and neuromuscular disorders.

Classification

Various diseases that present with a restrictive pattern on pulmonary function tests include:

Spirometry Findings in Various Lung Conditions

Spirometry can help distinguish restrictive lung disease from obstructive lung diseases. On spirometry the findings include:[1][2]

Pulmonary Function Test Obstructive Lung Disease Restrictive Lung Disease
Spirometry showing Obstructive and Restrictive Lung Disease ([Source:By CNX OpenStax [CC BY 4.0 (http://creativecommons.org/licenses/by/4.0)], via Wikimedia Commons])
TLC
RV
FVC
FEV1 ↓↓
FEV1/FVC N to
MVV

Approach to Lung Disorders

 
 
 
 
 
 
 
 
 
 
 
 
 
Spirometry
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Low FEV1/FVC ratio
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal to high FEV1/FVC ratio
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Obstructive Lung Disease
 
 
 
 
 
 
 
 
 
 
 
 
 
Restrictive Lung Disease
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Bronchodilator therapy
 
 
 
 
 
 
 
 
 
 
 
 
 
DLCO
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Increased FEV1
 
 
 
 
 
 
 
No change in FEV1
 
 
 
Normal DLCO
 
 
 
 
 
 
 
Decreased DLCO
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Asthma
 
 
 
 
 
 
 
COPD
 
 
 
Chest wall disorders
 
 
 
 
 
 
 
Interstitial Lung Disease
 
 

Differentiating Restrictive Lung Disease from other Diseases

Restrictive lung disease must be differentiated from other diseases that cause dyspnea, cough, hemoptysis, and fever such as ARDS, hypersensitivity pneumonitis, pneumoconiosis, sarcoidosis, pleural effusion, interstitial lung disease (ILD), lymphocytic interstitial pneumonia, obesity, pulmonary eosinophilia and neuromuscular disorders.

Disease Clinical manifestations Diagnosis
History Symptoms Physical exam Lab findings Pulmonary tests Imaging Gold standard of diagnosis
History/Exposure Dyspnea Cough Hemoptysis Fever Cyanosis Clubbing JVD Peripheral edema Auscultation Other prominent findings DLCO CXR CT Method
Acute Respiratory Distress Syndrome (ARDS)[3] + +/- - - +/- - +/- -
  • Bilateral pulmonary infiltrates
    • Initially patchy peripheral
    • Later diffuse bilateral
  • Ground glass
  • Frank alveolar infiltrate
  • Bronchial dilatation within areas of ground-glass opacification
  • PaO2 / FiO2 <300
Hypersensitivity Pneumonitis[4] + + - + - + - -
  • Constitutional symptoms
  • Poorly defined micronodular or diffuse interstitial pattern
  • In chronic form
    • Fibrosis
    • Loss of lung volume
    • Coarse linear opacities
  • Ground-glass opacities or
  • Diffusely increased radiodensities
  • Diffuse micronodules
  • Focal air trapping
  • Mosaic perfusion
  • Occasionaly thin-walled cysts
  • Mild fibrotic changes 
  • Clinical diagnosis
Pneumoconiosis[6] Silicosis[7][8]
  • Occupational history
    • Sandblasting
    • Bystanders
    • Quartzite miller
    • Tunnel workers
    • Silica flour workers
    • Workers in the scouring powder industry
+ + +/- - + + + -
  • Small round opacities
    • Symmetrically distributed
    • Upper-zone predominance
  • Diffuse interstitial pattern of fibrosis without the typical nodular opacities in chronic case
  • Nodular changes in lung parenchyma
  • Progressive massive fibrosis
  • Bullae, emphysema
  • Pleural, mediastinal, and hilar changes
Asbestosis[9]
  • Shipyard workers
  • Pipe fitting
  • Insulators
+ + +/- - + + + -
  • Predilection to lower lobes
  • Fine and coarse linear, peripheral, reticular opacities
  • Subpleural linear opacities seen parallel to the pleura
  • Basilar lung fibrosis
  • Peribronchiolar, intralobular, and interlobular septal fibrosis;
  • Honeycombing
  • Pleural plaques.
Berylliosis[10] 
  • Electronic manufactures
+ + +/- - + + + - ----
  • Hilar adenopathy
  • Increased interstitial markings.
  • Ground glass opacification
  • Parenchymal nodules
  • Septal lines
Byssinosis [11]
  • Cotton wool workers
+ + +/- - + + + -
  • Diffuse air-space consolidation
  • Pulmonary fibrosis with honeycombing
  • Peri bronchovascular distribution of nodules
  • Ground-glass attenuations
Sarcoidosis (stage 2-5)[12] + + + + - - - -
  • Usually normal
  • Occasional crackles
  • High-resolution CT (HRCT) scanning of the chest may identify
    • Active alveolitis
    • Fibrosis
Pleural Effusion Transudate

Exudate

+ + +/- +/- +/- +/- +/- +/-
  • Peripheral edema, distended neck veins, and S3 gallop suggest congestive heart failure.
  • Edema may also be a manifestation of nephrotic syndrome, pericardial disease, or, when combined with yellow nailbeds, the yellow nail syndrome.
  • Cutaneous changes and ascites suggest liver disease.
  • Lymphadenopathy or a palpable mass suggests malignancy.
N Supine
  • Blunting of the costophrenic angle
  • Homogenous increase in density spread over the lower lung fields

Lateral decubitus

  • Free flowing effusion as layers
  • Thickened pleura
  • Mild effusions can aslo be detected
Interstitial lung disease[14] ++ + + - +/- +/- +/- +/-
  • Increased A-a gradient
  • Normal PCO2
Depending on the underlying cause:
  • Elevated ESR
  • Serologic testing for ANA, RF, ANCA & ASCA may be positive
  •  Reticular and/or nodular opacities
  • Honeycomb appearance (late finding)
  • Bilateral reticular and nodular interstitial infiltrates
Video-assisted thoracoscopic lung biopsy
Lymphocytic Interstitial Pneumonia[15] + + + + - + - -
  • Increased A-a gradient
  • Normal PCO2
  • Polyclonal hypergammaglobulinemia
  • Increased LDH
N
  • Bibasilar interstitial or micronodular infiltrates
  • Determines the degree of fibrosis
  • Cysts (characterstic)
Open lung biopsy
Obesity[16][17] + + - - - - - + - N
  • X ray findings are often limited due to body habitus
  • CT findings are variable and depends upon severity of obesity
Clinical
Pulmonary Eosinophilia[18] Infections + + + + + - + +
  • Increased A-a gradient
  • Interstitial or diffuse nodular densities
  • Determines extent and distribution of the disease
  • Interstitial infiltrates
  • Cysts and nodules
Biopsy of lesion (skin or lung)
Neuromuscular disease[19] Scoliosis[20]
  • Postural abnormality
+ - - - - - - -
  • In severe scoliosis, the rib cage may press against the lungs making it more difficult to breathe.
N
  • Accurate depiction of the true magnitude of the spinal deformity can be assessed by supine anteroposterior (AP) and lateral spinal radiographs
  • N/A
  • Clinical
  • Radiographs
Muscular dystrophy
  • Proximal muscle weakness
+ - - - - - - - N
  • N/A
  • N/A
ALS[21]
  • Muscle weakness
  • Neurological deficit
+ - - - - - - -
  • Symptoms begin with limb involvement diue to muscle weakness and atrophy. 
  • Cognitive or behavioral dysfunction
  • Sensory nerves and the autonomic nervous system are generally unaffected
N/A N Not significant/diagnostic Not significant/diagnostic
Myasthenia gravis[22] H/O of difficulty getting up from chair + - - + - - - -
  • Extraocular, bulbar, or proximal limb muscles.
  • Breathing as rapid and shallow
  • Respiratory muscle weakness can lead to acute respiratory failure may require immediate intubation.
  • Anti–acetylcholine receptor (AChR) antibody (Ab) test +
N
  • Thymoma as an anterior mediastinal mass.
  • Thymoma as an anterior mediastinal mass.

References

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