This summary contains information on the general characteristics, aetiology, pathology and pathogenesis of Restrictive Pulmonary Diseases. It clearly defines all subcategories of the aforementioned disease within the scope of the sources used. It also distinguishes between obstructive and restricti...
Sources
● Kumar, et al.- Kumar and Clark’s Clinical Medicine (8thed., 2012): Chapter 15
● Jameson, et al.- Harrison's Principles of Internal Medicine (20th ed., 2018): Chapters
2, 9 & 13
Introduction
● What are restrictive diseases?
➔ They are diseases characterised by impaired airway filling due to
fibrosis of alveolar septae
● General Characteristics
➔ Restrictive = ↓Compliance, ↑Elastance→ Problem getting air in.
➔ ↓DLCO in ILD. Normal DLCO in neuromuscular disorders.
➔ ↑DLCO in obesity, ↓TLC, ↓RV.
➔ Increase in radial traction, so tethered more strongly and airways
remain open
➔ FEV1↓, FVC↓↓ → FEV1/FVC ratio normal or increased
● Restrictive vs Obstructive
➔ FEV1 and FVC ratio are decreased in obstructive whereas normal in
restrictive
Figure 1: Etiology and types of obstructive diseases vs restrictive diseases
, 3
Figure 2: Pathophysiological differences between obstructive diseases and restrictive
diseases.
● Types
➔ Extra pulmonary (Characteristics and causes)
➢ Characterised by external impairment of lung filling
➢ Can be caused by:
1. Conditions affecting the chest wall such as
kyphoscoliosis, obesity hypoventilation syndrome
(AKA Pickwickian syndrome in people with BMI >50,
hypoventilation is as a result of decreased central drive
to breathe)
2. Diaphragmatic disease
3. Neuromuscular diseases: e.g. Guillain-Barré,
poliomyelitis and past polio.
➔ Intrapulmonary (Definition, characteristics and types)
Intrapulmonary restrictive diseases
● Introduction
➔ They account for 15% of respiratory failure
➔ It involves diffuse lung injury and inflammatory responses that can
lead to lung fibrosis
● General Characteristics
➔ Thickened alveolar interstitium as a result of accumulation of
inflammatory cells, mesenchymal cells and collagen-rich extracellular
matrix
➔ Fibrosis of lung parenchyma and capillary remodelling
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