Restrictive lung disease

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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 (RLD) is a group of diseases characterized by an inability to attain complete expansion of the lungs. This may result from an abnormality in the parenchyma of the lungs or an abnormality outside the lung parenchyma (such as fluid accumulation or musculoskeletal abnormalities) hindering normal lung expansion and thus ability to ventilate normally. Restrictive lung diseases presents with a restrictive pattern on pulmonary function test and includes a decrease in total lung capacity (TLC), residual volume (RV), forced vital capacity (FVC), forced expiatory volume (FEV1), and a normal to increased FEV1/FVC ratio. Unlike obstructive lung disease (characterized by air trapping within the lungs), restrictive lung diseases result in decreased lung volumes and a lower than normal amount of air within the lungs. RLDs 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 gravis.

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 Tests (PFT) 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])
Total lung capacity (TLC)
Residual volume (RV)
Forced vital capacity (FVC)
Forced expiratory volume

in 1st second

(FEV1)

↓↓
FEV1/FVC ratio N to
Maximum voluntary ventilation

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 PFT Imaging Gold standard
History/Exposure Dyspnea Cough Hemoptysis Fever Cyanosis Clubbing JVD Peripheral edema Auscultation Other prominent findings DLCO CXR CT
Acute Respiratory Distress Syndrome (ARDS)[3] + +/– +/– +/–
  • Bilateral pulmonary infiltrates:
    • Initially patchy peripheral
    • Later diffuse bilateral
  • Ground glass appearance
  • 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
  • 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
  • Lung biopsy
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
Disease Clinical manifestations Diagnosis
History Symptoms Physical exam Lab findings PFT Imaging Gold standard
History/Exposure Dyspnea Cough Hemoptysis Fever Cyanosis Clubbing JVD Peripheral edema Auscultation Other prominent findings DLCO CXR CT
Sarcoidosis

(stage 2–5)[12]

+ + + +
  • Usually normal
  • Occasional crackles
  • High–resolution CT (HRCT):
    • Active alveolitis
    • Fibrosis
Pleural Effusion Transudate

Exudate

+ + +/– +/– +/– +/– +/– +/– NL 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, and 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
NL
  • Bibasilar interstitial or micronodular infiltrates
  • Determines the degree of fibrosis
  • Cysts (characterstic)
  • Open lung biopsy
Obesity[16][17] + + + NL
  • 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)
Disease Clinical manifestations Diagnosis
History Symptoms Physical exam Lab findings PFT Imaging Gold standard
History/Exposure Dyspnea Cough Hemoptysis Fever Cyanosis Clubbing JVD Peripheral edema Auscultation Other prominent findings DLCO CXR CT
Neuromuscular diseases[19] Scoliosis[20]
  • Postural abnormality
+
  • In severe scoliosis, the rib cage may press against the lungs making it more difficult to breathe.
NL
  • 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
+ NL
  • N/A
  • N/A
ALS[21] +
  • N/A
NL
  • Not significant
  • Not significant
Myasthenia gravis[22]
  • Difficulty in:
+ +
  • Extraocular, bulbar, or proximal limb muscles.
  • Breathing is rapid and shallow
  • Respiratory muscle weakness may lead to acute respiratory failure and require immediate intubation
  • Anti–acetylcholine receptor (AChR) antibody (Ab) test positive
NL
  • Thymoma as an anterior mediastinal mass
  • Thymoma as an anterior mediastinal mass

References

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