Restrictive Lung Disorder: Types, Causes and Treatment

Restrictive lung diseases are characterised by reduced lung volumes which can be due to alteration in lung parenchyma or because of disease to the pleura, chest wall or any neuromuscular system. The intrinsic lung diseases can cause inflammation or scarring of the lung tissue (Interstitial lung disease). Most of the causes of intrinsic lung diseases are occupational and environmental. The extrapulmonary diseases like neuromuscular disorders affecting the chest wall result in lung restriction and respiratory failure.

Restrictive Lung Diseases Types and Causes

Parenchymal  Lung Diseases (Interstitial Lung Diseases)

• Pneumoconiosis (Coal workers pneumoconiosis, Silicosis, asbestosis)
• Sarcoidosis
• Idiopathic pulmonary fibrosis
• Granulomatosis with polyangitis
• Pulmonary Langerhans cell with histiocytosis
• Hypersensitivity pneumonitis
• Drug toxicity (Bleomycin, Busulfan)
• Acute respiratory distress syndrome
• Reduced-induced Lung injury

Extrapulmonary Causes

• Respiratory muscle weakness ( Polio, myasthenia gravis, Guillain-Barre syndrome , ALS)
• Chest wall abnormalities (Scoliosis, severe obesity)

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Restrictive Lung Diseases Symptoms and Clinical Features

• Patient will be presented as shortness of breath , difficulty breathing and feeling of suffocation
• Wheezing
• Coughing and constant clearing of the throat
• Chest Pain
• Fatigue: There will be extreme tiredness due to regular exertion.
• Anxiety
• Depression

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Restrictive Lung Diseases Diagnosis

Spirometry is done and the flow-volume loops are studied to diagnose and differentiate it from Obstructive lung diseases like Asthma.

Findings of Flow-Volume Loops

• Decreased Residual volume (RV)
• Decreased Functional Residual Capacity (FRC)
• Decreased Total Lung Capacity (TLC)
• Decreased FEV1
• Decreased FVC
• There will be normal or increased FEV1/FVC

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Important Restrictive Lung Diseases

Idiopathic Pulmonary Fibrosis

• It is the progressive fibrotic lung disease and It has not any defined etiology. 
• It involves the chronic insult to the lung and there are multiple cycles of lung injury, inflammation and fibrosis. 
• Its main association is with frequent exposure to ciggratte smoke or environmental pollution.
• The patient will present with progressive dyspnea, fatigue, crackles and clubbing.
• On Chest Xray: There will be honey comb appearance of the lung with peripheral reticular opacities.
• On Histology: It will present the Interstitial Pneumonia.

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Hypersensitivity Pneumonitis

• It is often seen in farmers and bird fanciers. It is a type of Hypersensitivity reaction to the environmental antigens.
• The findings will be dyspnea, cough, chest tightness, fever and headache.
• It is a self limiting disease but chronically it can lead to irreversible fibrosis with noncaseating granuloma, alveolar septal thickening and traction bronchiectasis.

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Sarcoidosis

• It is an autoimmune disease which can affect multiple organs of the body and lungs are the most important parts to get affected.
• The main characteristic points are presence of widespread noncaseating granuloma, elevated serum ACE levels and elevated CD4/CD8 ratio in the Bronchoalveolar lavage. 
• Very common in black females.
• Chest Xray will present as Bilateral adenopathy and coarse reticular opacities. CT chest is better.
• Other associated diseases are Bell’s palsy, Uveitis, hypercalcemia and erythema nodosum.
• Treatment is steroids

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Pneumoconiosis

• Asbestosis- It is a type of pulmonary fibrosis caused due to exposure to asbestos.
  • The other associated problems will be pleural disease and malignancies.
  • The main reason for asbestos exposure is occupation at roofing , plumbing and shipbuilding.
  • On Chest Xray, there will be presence of calcified, supradiaphragmatic and pleural plaques.
  • It mainly affects the lower lobes and there is increased risk of bronchogenic carcinoma (more chances) and mesothelioma (less chances) .
  • On visualizing the Asbestos bodies on BAL with prussian blue stain the golden-brown dumbbells like structures will be found .
• Berrylliosis 
  • It is associated with exposure to beryllium and it occurs in people who work in aerospace and manufacturing industries.
  • Affects the upper lobes mainly.
  • On histology, Non- Caseating granulomatous are seen.
• Coal workers pneumoconiosis
  • Associated with prolonged exposure to the coal dust and that leads to black lung disease.
  • Mainly affects the upper lobes
  • Small, rounded nodular opacities seen on imaging.
  • The dust particles get trapped in to the macrophages and then the further inflammation and fibrosis will occur.
• Silicosis
  • It is associated with sandblasting, foundries and mines.
  • Affects the upper lobes and on Chest xrays, egg-shell calcification of hilar lymoh nodes are found.
  • The patients will have high susceptibility to TB. Macrophages respond to silica and release fibrogenic factors leading to fibrosis.

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Radiation Induced Lung Injury

•  It is associated with proinflammatory cytokine release ( TNF alpha, IL-1, IL-6) .
• Most common symptoms are dry cough and dyspnea.

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