Interstitial and Occupational Lung Disease Flashcards
What does interstitial disease interfere with and what is the lung pattern?
Interferes with gas transfer
Restrictive lung pattern
Give two symptoms of interstitial disease?
Breathlessness and dry cough
What class of interstitial lung disease is part of systemic disease, exposure to agent or idiopathic?
Chronic interstitial lung disease
Name two granulomatous interstitial lung diseases?
Sarcoidosis
Extrinsic allergic alveolitis
Name a granulomatous type 4 hypersensitivity disease of unknwon cause?
Sarcoidosis
What are the 6 common system involvements in sarcoidosis?
- Lungs
- Lymph nodes
- Joints
- Liver
- Skin
- Eyes
What type of granuloma does sarcoidosis have?
Non-caseating
What patients is sarcoidosis less common in?
Smokers
What are the two types of sarcoidosis?
Acute
Chronic
Give 5 clinical features of acute sarcoidosis?
Erythema nodosum Bilateral hilar lymphadenopathy Arthritis Uveitis, parotitis Fever
Give 5 clinical features of chronic sarcoidosis?
Lung infiltrates (alveolitis) Skin infiltrations Peripheral lymphadenopathy Hypercalcaemia Other organs: renal, myocardial, neurologicalm hepatitis, splenomegaly
Give four differential diagnosises of sarcoidosis?
TB (tuberculin test -ve)
Lymphoma
Carcinoma
Fungal infection
On a CXR of sarcoidosis what will you see?
Bilateral hilar lymphadenopathy
On a CT scan of lungs with sarcoidosis, what might you see?
Peripheral nodular infiltrate
What would you look for in a tissue biopsy of sarcoidosis?
Non-caseating granuloma
In pulmonary function of sarcoidosis, what causes the restrictive defect?
Lung infiltrates
With a blood test for sarcoidosis, what 3 things would suggest diagnosis?
- Angiotensin Converting Enzyme (ACE) levels as activity marker
- Raised calcium
- Increased inflammatroy markers
What is the treatment for acute sarcoidosis?
Self-limiting condition = no treatment, steroids if vital organ affected
What is the treatment for chronic sarcoidosis?
Oral steroids
Immunosuppression (e.g. azathioprine, methotrexate, anti-TNF therapy)
What clinical feature of the eyes can suggest sarcoidosis?
Iritis
What is another term for extrinsic allergic alveolitis?
Hypersensitivity pneumonitis
What type of reaction is extrinsic allergic alveolitis?
Type III hypersensitivity (immune complex deposition) reaction to antigen
Give three causes of extrinsic allergic alveolitis?
- Thermophilic actinomycetes (farmers lung, malt workers, mushroom workers)
- Avian antigens (bird fanciers lung)
- Drugs (gold, bleomycin, sulphasalazine)
What does this description describe: cough, breathless, fever, myalgia, symptoms occur several hours after acute exposure, signs (+/- pyrexia, crackles, no wheeze, hypoxia) and on CXR widespread pulmonary infiltrates?
Acute extrinsic allergic alveolitis
What is the treatment for acute extrinsic allergic alveolitis?
Oxygen
Steroid
Antigen avoidance
What causes chronic extrinsic allergic alveolitis?
Repeated low dose antigen exposure over time (years)
What condition does this describe: Progressive breathlessness and cough, signs (may be crackles, clubbing is unusual), CXR = pulmonary fibrosis - most commonly in the upper zones?
Chronic extrinsic allergic alveolitis
What will the pulmonary function tests be like in chronic extrinsic allergic alveolitis?
Restrictive defect (Low FEV1 & FVC, high or normal ratio, low gas transfer - TLCO)
What three factors do you use to diagnose chronic extrinsic allergic alveolitis?
History of exposure
Precipitins (IgG antibodies to guily antigen, lung biopsy if in doubt)
What treatment is there for chronic extrinsic allergic alveolitis?
Remove antigen exposure, oral steroids if breathless or low gas transfer
What is formerly known as cryptogenic fibrosing alveolitis?
Idiopathic pulmonary fibrosis
Is idiopathic pulmonary fibrosis an inflammatory disease?
No
N.B More common in smokers
What are rheumatoid, SLE, systemic sclerosis, asbestos, (drugs - amiodarone, busulphan, bleomycin, penicillamine, nitrofurantoin, methotrexate all secondary causes of?
Pulmonary fibrosis
What would you find on clinical presentation of idiopathic pulmonary fibrosis?
Progressive breathlessness (several years) and dry cough
What would you find on examination of idiopathic pulmonary fibrosis?
Clubbing
Bilateral fine inspiratory crackles
What would you find on investigation of idiopathic pulmonary fibrosis, including PFTs?
Restrictive defect on PFTs - reduced FEV1 and FVC with normal or raised FEV1/FVC ratio, reduced lung volumes and low gas transfer
What would you see on CXR of idiopathic pulmonary fibrosis?
Bilateral infiltrates
What might you see on a CT scan in idiopathic pulmonary fibrosis?
Reticulonodular fibrotic change, worse at the lung bases. The presence of “ground-glass” suggests reversible alveolitis; fibrosis is irreversible.
Give 4 differential diagnosis of idiopathic pulmonary fibrosis?
- Mitral valve disease
- Left ventricular failure
- Sarcoidosis
- Extrinsic allergic alveolitis
Describe the pathology of idiopathic pulmonary fibrosis?
Chronic inflammatory infiltrate (neutrophils and fibrosis in alveolar walls +/- intra-alveolar macrophages)
What is coal workers pneumoconiosis?
Simple pneumoconiosis
What is there an abnormality with in coal workers pneumoconiosis?
CXR (no impairment of lung function - often associated with chronic obstructive pulmonary disease)
What does complicarted pneumoconiosis lead to?
Progressive massive fibrosis = restrictive pattern with breathlessness
What is Caplan’s syndrome?
Rheumatoid pneumoconiosis (pulmonary nodules)
What causes silicosis?
15-20 years exposure to quartz (e.g. mining, foundry workers, glass workers, boiler workers)
What disease has egg-shell calcification of hilar nodes?
Silicosis
What lung pattern is present in chronic silicosis?
Restrictive pattern
What do mining, construction, shipbuilding, boilers and piping and automotive components like brake linings all have in common?
Asbestos exposure
Name 4 features of pleural disease caused by asbestos?
- Benign pleural plaques - asymptomatic
- Acute asbestos pleuritis - fever, pain, bloody pleural effusion
- Pleural effusion and diffuse pleural thickening - restrictive impairment
- Maligannt mesothelioma - incurable pleural cancer.
How does malignant mesothelioma present?
With chest pain and pleural effusion
What does heavy, prolonged exposure of asbestos cause?
Asbestosis - pulmonary fibrosis
Where can you find asbestos in a patient with asbestosis?
Asbestos bodies in sputum
Asbestos fibres in lung biopsy
What is interstitial disease?
Any disease process affecting lung interstitium (i.e. alveoli, terminal bronchi)
What is a thin elastin-rich connetive componenet containing capillary blood vessels?
Pulmonary interstitium
What cells are present in pulmonary interstitium?
Alveolar lining cells
What are the two stages in interstitial lung disease?
- Early stage is alveolitis (injury with inflammatory cell infiltration)
- Late stage characterised by fibrosis
What are the two biopsies in interstitial lung disease?
Transbronchial biopsy - special forceps used at bronchoscopy
Thoracoscopic biopsy - more invasive but more reliable and generates far more tissue
Name 5 chronic intersitial diseases?
- Fibrosing alveolitis
- Sarcoidosis
- Extrinsic allergic alveolitis (hypersensitivity pneumonitis)
- Pneumoconiosis
- Connective tissue disease
What involves progressive interstitial fibrosis or unknown cause, variable associated inflammation and finger clubbing?
Fibrosing alveolitis
What are three pathalogical features of fibrosing alveolitis?
Subpleural and basal fibrosis
Inflammatory component variable
Terminally lung structure replaced by dilated spaces surrounded by fibrous walls
What condition does late honeycombing on histology appear in?
Fibrosing alveolitis
Extrinsic allergic alveolitis can either be a chronic inflammatory disease or allergic origin - what are the three features of chronic inflammatory disease?
Small airways
Interstitium
Occasional granulomas
What does thermophilic bacteria cause?
Farmer’s lung
What do avian proteins cause?
Bird fanciers lung
What does fungi cause?
Malt workers lung
What is uveitis (occurs with sarcoidosis)?
Inflammation of iris
Name 3 pulmonary involvements in connective tissue diseases?
- Interstitial fibrosis (milder than fibrosing alveolitis)
- Pleural effusions
- Rheumatoid nodules
What is pneumoconiosis? Give examples?
Lung disease caused by mineral dust exposure. Examples include asbestosis, coal workers lung and silicosis
What is asbestos?
A silicate
What asbestos fibres are safe and which ones are dangerous?
Serpentine (curved) asbestos fibres are safe
Straight (amphibole) asbestos highly dangerous
What four things can asbestos cause?
- Parietal pleural plaques
- Interstitial fibrosis (asbestosis)
- Bronchial carcinoma
- Mesothelioma