Lecture 11.0 - Restrictive Lung Disease Notes Flashcards
Name the 2 sites where the causes of interstitial fibrosis mostly occur in the lung
- At the lower lung
- Sparing of the lower lung/lung bases (occur not in these regions)
Name the causes of interstitial fibrosis occuring at the lower lung
- Organising DAD
- Acute interstitial fibrosis (Hamman-Rich)
- Chronic idiopathic pulmonary fibrosis:
a.Primary
b.Secondary - ie with systemic disease (ie rheumatoid arthritis, systemic sclerosis) - Chronic oedema
- Asbestosis
Name the causes of interstitial fibrosis sparing the lower lung
- Sarcoidosis
- Extrinsic allergic alveolitis /
hypersensitivity pneumonia - Silicosis
- Chronic berylliosis
- Infections with a granulomatous pattern
(may involve the whole lung, ie TB)
What are the 2 clinical presentations of DAD?
- Hypoxia (low oxygen levels)
- Bilateral lung opacities on chest imaging
ALI / ARDS / DAD (Diffused Alveolar Damage)
Same disease process just different names used in different departments
PATHOGENESIS OF ADA:
Name the 2 stages of ADA
- Early / exudative
○ Endothelial activation
○ Adhesion and extravasation of neutrophils
○ Accumulation of intra-alveolar fluid and formation of hyaline
membranes - Organising / proliferative
-Type 2 pneumocyte hyperplasia
-Increase of fibroblasts and myofibroblasts within the alveoli wall
- Interstitial inflammation and fibrosis
● Chronic or fibrotic stage which resolves the injury
PATHOLOGIC FINDINGS OF DAD:
What are the expected findings of DAD macroscopically?
Heavy, firm, red, boggy lung
What are the characteristics of DAD survivors?
Survivors:
- Recover, some may have persistent impairment in cognitive and physical function
- Chronic lung disease : scarring, interstitial fibrosis, end stage/honeycomb lung
Restrictive Lung Diseases are classified according to the anatomical occassion that is affected. What 2 anatomical occassions are normally affected on RLD?
- Chest wall disorders
These include:
-Neuromuscular diseases
incases of-
○ Poliomyelitis
○ Severe obesity
○ Pleural disease
○ Kyphoscoliosis - Chronic diffuse ‘interstitial diseases’ of the lung parachyma
These include:
-Fibrosing diseases
○ Granulomatous disorders
○ Smoking-related interstitial diseases
○ Other:
■Langerhans cell histiocytosis
■Pulmonary alveolar proteinosis
■Lymphoid interstitial pneumonia
PATHOLOGIC FINDINGS OF DAD:
What are the expected findings of DAD microscopically?
Early stage:
- Congestion
- Interstitial and intra-alveolar oedema
- Fibrin deposition
- Waxy hyaline membranes
- Fibrin-rich oedema fluid
- Cytoplasmic and lipid remnants of necrotic epithelial cells
Organising stage:
- Proliferation of type II pneumocytes
- Granulation tissue in alveolar walls and spaces
- Fibrous scarring may occur, not always
* May have superimposed bronchopneumonia
What do the patients hospitalized for DAD normaly present with?
Patients usually hospitalised for ALI:
- Dyspnoea, tachypnoea, cyanosis, hypoxaemia, respiratory failure
- Radiology : diffuse bilateral infiltrates
- Ventilation required : V/Q mismatch (uneven distribution of injured lung, some unaerated and collapsed, other regions almost normal), respiratory acidosis
Name the conditions or disoders that fall under fibrosing diseases
● Usual interstitial pneumonia
● Non-specific interstitial pneumonia
● Cryptogenic organising pneumonia
● Connective tissue disease-associated
● Pneumoconiosis
○Silicosis
○Asbestosis
○Coal workers pneumoconiosis
● Drug reactions
● Radiation pneumonia
What is pneumoconiosis?
● Non-neoplastic lung reaction to mineral dusts, particulates, chemical fumes and vapours in the workplace
● It is an occupational lung disease
● Examples of mineral dusts:
○ Silica - gold miners, stone workers, sandblasters
○ Asbestos : asbestos mines, ship builders, insulation, electrical work
○ Coal
Discuss the pathogenesis of pneumoconiosis
- Amount of dust retained in lung and airways
a. Dust concentration duration of exposure, effectiveness of clearing mechanisms - Size, shape and buoyancy of the particles
a. Most dangerous particles : 1 -5 μm in diameter - Particle solubility and physiochemical properties
a. Small particles of high solubility may cause rapid onset lung damage
b. Larger particles may resist dissolution - Possible additional effects of other irritants
a. Tobacco smoke
What is silicosis?
Common lung disease caused by inhalation of silica (crystallin silicon dioxide)