interstitial lung disease prof. fa carey. pulmonary interstitium r alveolar lining cells (types 1...
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Interstitial Lung DiseaseInterstitial Lung DiseaseInterstitial Lung DiseaseInterstitial Lung Disease
Prof. FA Carey
Pulmonary interstitiumPulmonary interstitium
Alveolar lining cells (types 1 and 2)
Thin elastin-rich connective component containing capillary blood vessels
Interstitial lung diseaseInterstitial lung disease
Early stage is alveolitis (injury with inflammatory cell infiltration)
Late stage characterised by fibrosis
Clinical effects due to hypoxia (respiratory failure) and cardiac failure
Causes….Causes….
Environmental (minerals, drugs, radiation. Post-ARDS) Hypersensitivity (mouldy hay, avian proteins)
Unknown (idiopathic) Connective tissue diseases Fibrosing alveolitisDiagnosis based on clinical features often with
biopsy
Biopsy in interstitial lung disease…Biopsy in interstitial lung disease…
Transbronchial biopsy – special forceps used at bronchoscopy
Thoracoscopic biopsy – more invasive but more reliable and generates far more tissue
Chronic Chronic Interstitial DiseaseInterstitial Disease
Fibrosing alveolitis Sarcoidosis Extrinsic allergic alveolitis
(hypersensitivity pneumonitis) Pneumoconiosis Connective tissue diseases
Fibrosing AlveolitisFibrosing Alveolitis
aka cryptogenic fibrosing alveolitis (CFA), usual interstitial pneumonia (UIP)
Progressive interstitial fibrosis of unknown cause
Variable associated inflammation Finger clubbing
PathologyPathology
Subpleural and basal fibrosis Inflammatory component variable Terminally lung structure replaced by
dilated spaces surrounded by fibrous walls
Fibrosing alveolitis (early)Fibrosing alveolitis (early)
Fibrosing alveolitis (late – Fibrosing alveolitis (late – honeycombing)honeycombing)
Honeycombing – Honeycombing – basal/subpleural (a splint!)basal/subpleural (a splint!)
Extrinsic allergic alveolitis Extrinsic allergic alveolitis (hypersensitivity pneumonitis)(hypersensitivity pneumonitis)
Chronic inflammatory disease
Small airways Interstitium Occasional granulomas
Allergic origin Type III
hypersensitivity Type IV
hypersensitivity
EAA – inflammatory interstitial EAA – inflammatory interstitial expansionexpansion
EAA - granulomaEAA - granuloma
Causes of EAACauses of EAA
Thermophilic bacteria – Farmers lung Avian proteins – Bird fanciers lung Fungi – Malt workers lung
Precipitins (antibodies) often detectable in serum. Unusual cases come to biopsy.
SarcoidosisSarcoidosis
Multisystem granulomatous disorder of unknown cause (defined by histological means)
Pulmonary involvement is common Most cases mild and self-limiting
Other manifestations of Other manifestations of sarcoidosissarcoidosis
Uveitis (inflammation of iris) Erythema nodosum Lymphadenopathy Hypercalcaemia
Transbronchial biopsy - sarcoidosisTransbronchial biopsy - sarcoidosis
Sarcoidosis - granulomaSarcoidosis - granuloma
Apical scarring in sarcoidosisApical scarring in sarcoidosis
Sarcoid – scarring and burnt-out Sarcoid – scarring and burnt-out granulomasgranulomas
Sarcoid - granulomasSarcoid - granulomas
Pulmonary involvement in connective Pulmonary involvement in connective tissue diseases tissue diseases
Interstitial fibrosis (milder than fibrosing alveolitis)
Pleural effusions Rheumatoid nodules
Rheumatoid noduleRheumatoid nodule
Rheumatoid noduleRheumatoid nodule
PneumoconiosisPneumoconiosis
Lung disease caused by mineral dust exposure
• Asbestosis• Coal workers lung• Silicosis
Thin whole mount section of a coal-Thin whole mount section of a coal-workers lung (unstained)workers lung (unstained)
Coal miner with progressive massive Coal miner with progressive massive fibrosis (unstained)fibrosis (unstained)
Disease depends on….Disease depends on….
Particle size (1-5m) Reactivity of particle Clearance of particle Host response
Asbestos Asbestos
A silicate Serpentine (curved) asbestos fibres
relatively safe Straight (amphibole) asbestos highly
dangerous
Asbestos…Asbestos…
Parietal pleural plaques Interstitial fibrosis (asbestosis) Bronchial carcinoma Mesothelioma
Asbestosis bodies (from human Asbestosis bodies (from human lung)lung)
Association of asbestos bodies with Association of asbestos bodies with fibrosis (asbestosis)fibrosis (asbestosis)
Pleural plaque on diaphragmPleural plaque on diaphragm