Interstitial Lung Diseases Flashcards
what is interstitial lung disease?
– Lung parenchymal disorders with common clinical, radiologic, physiologic and pathologic features; hallmark – Involvement of interstitium
synonyms of interstitial lung disease?
• Infiltrative lung disease -Infiltration of cellular and non-cellular
elements within alveolar septa and alveoli.
• Diffuse parenchymal lung disease (DPLD)
• Restrictive lung disease – Characterized by reduced total lung capacity in presence of a normal or reduced expiratory flow rate.
types of interstitial lung disease
• Acute interstitial lung disease – Acute interstitial pneumonia (ARDS)
• Chronic interstitial lung disease
– Fibrosing lung disorders (pneumoconioses)
– Granulomatous disorders (sarcoidosis)
– Idiopathic interstitial pneumonias (IIPs)
pathogenesis of “alveolitis”?
– Damage to pneumocytes and endothelial cells
• Leads to leukocytes releasing cytokines which mediate and stimulate interstitial fibrosis
• Interstitial fibrosis –
– ↓es lung compliance and elasticity
• ↓ed lung expansion during inspiration
clinical laboratory findings of interstitial lung disease
- Dry cough and dyspnea
- Late inspiratory crackles, bibasilar (Velcro crackles)
- Cor pulmonale
- Chest radiography – bilateral reticulonodular infiltrates
Pneumoconioses definition
Non- neoplastic lung diseases in response to inhalation of mineral dusts inhaled in the workplace.
• Now expanded to include diseases induced by organic and inorganic particulate matter/chemical fumes /vapors.
• Coal dust, silica, asbestos, beryllium
what percent of interstitial lung disease does pneumoconiosis cause?
• 25% cases of chronic interstitial lung disease
what does the development of pneumoconiosis depend upon?
• The amount of dust retained in the lung parenchyma and airways
• The size, shape and buoyancy of particles
– 1 – 5 μm – reach bifurcation of respiratory bronchioles and alveolar ducts
– < 0.5 μm – reach alveoli and are phagocytosed by alveolar macrophages (much worse!!)
• Particle solubility and physicochemical reactivity
• Possible additional effects of other irritants (e.g., tobacco smoking)
causes of fibrogenic pneumoconiosis
- coal worker’s pneumoconiosis (carbonaceous dust)
- silicosis (crystalline silica)
- silicatosis (talc, kaolin, fedspar, mica, muscovite)
- asbestosis (asbestos fibers)
- rare forms:
- metalloconiosis
- berylliosis (beryllium dust)
- hard metal lung disease (tungsten carbide)
- aluminosis (aluminum)
- thesaurosis (hair spray)
- metalloconiosis
types of Coal workers’ pneumoconiosis (CWP)
- Anthracotic pigment – coal mines, urban centers, tobacco smoke
- Pulmonary anthracosis
- Simple CWP
- Complicated CWP (progressive massive fibrosis)
Anthracosis findings
- Asymptomatic
* Anthracotic pigment in interstitial compartment and lymph nodes
findings of Simple coal workers’ pneumoconiosis
- Fibrous opacities < 1 cm
- Upper lobes and upper portions of lower lobes
- Characterized by coal dust deposits adjacent to respiratory bronchioles
findings of Complicated coal workers’ pneumoconiosis (progressive massive fibrosis)
• Fibrous opacities > 1 cm • With or without central necrosis • Massive fibrosis – crippling lung disease (Black lung disease) • Caplan syndrome – CWP with rheumatoid nodules in lung
complication of Complicated coal workers’ pneumoconiosis (progressive massive fibrosis)
• Complication – Cor pulmonale
does Complicated coal workers’ pneumoconiosis (progressive massive fibrosis) increase the risk of a patient developing TB or cancer?
• No increased incidence of TB or cancer.
prevalence of silicosis
• Most common occupational disease worldwide
cause of silicosis
• Crystalline silicon dioxide (quartz)
– Foundries (metal casting), sandblasting, silica mines
• Quartz activates alveolar macrophages after engulfment → cytokine release → fibrogenesis
what do you get from chronic exposure to silicosis?
– Nodular opacities with concentric layers of collagen
– Polarizable quartz particles can be seen
– “Egg-shell” calcification in hilar lymph nodes
complications of silicosis?
• Complications – cor pulmonale; association with Caplan syndrome
does silicosis increase the risk of a patient developing TB or cancer?
yes,
• Increased risk for TB (silicotuberculosis) and cancer
forms of asbestos
– Serpentine (e.g.–chrysotile)–curlyandflexible
– Amphibole (e.g. – crocidolite) – straight and rigid
deposition sites of asbestos-related disease
• Deposition sites – respiratory bronchioles, alveolar ducts and alveoli
sources of asbestos-related disease
• Sources –
– Insulation around pipes in old naval ships
– Roofing material used over 20 years ago
– Demolition of old buildings