Interstitial Lung Disease Flashcards
d:interstial lung disease
Any disease process affecting lung interstitium (ie alveoli, terminal bronchi).
symptoms of ILD
SOB
dry cough
what does ILD do?
interferes with gas transfer
restrictive lung pattern
what is sarcoidosis?
growth of tiny collections of inflammatory disease in lungs/lymph nodes but can occur anywhere
what type of hypersensitivity reaction is sarcoidosis and is it granulomatous?
4 and yes
describe the pathology of sarcoidosis
non-caseating granuloma
is sarcoidosis less/more common in smokers?
less
Name some symptoms of acute sarcoidosis
erythema nodosum bilateral hilar lymphadenopathy arthritis uveitis, parotitis fever.
give some symptoms of chronic sarcoidosis
lung infiltrates (alveolitis) skin infiltrations peripheral lymphadenopathy hypercalcaemia Other organs: renal, myocardial, neurological, hepatitis, splenomegaly
Name the tests for positive sarcoidosis and what they show
CXR- BHL CT- peripheral nodular infiltrate tissue biopsy- non-caseating granuloma LFT- restrictive function test BT- angiotension converting enzyme raised calcium increased inflammatory markers
treatment of sarcoid
Steroids if vital organ affected
if chronic oral steroids normally required
immunosuppression
continuous monitoring
d: Hypersensitivity pneumonitis (extrinsic allergic alveolitis)
It is an inflammation of the alveoli (airspaces) within the lung caused by hypersensitivity to inhaled organic dusts
what type of hypersensitivity reaction is hypersensitivity pneumonitis
3
what is the aetiology of HP?
Thermophilic actinomycetes (farmers lung, malt workers, mushroom workers), avian antigens (bird fanciers lung), drugs (gold, bleomycin, sulphasalazine
pathology of HP
hypersensitivity pneumonitis
symptoms of acute HP
cough, breathless, fever, myalgia –
Classically symptoms occur several hours after acute exposure (flu-like illness)
Signs: +/- pyrexia, crackles (no wheeze!), hypoxia
CxR: widespread pulmonary infiltrates
Treatment of HP
oxygen, steroid and antigen avoidance
Anti-fibrotic therapy in cases of progressive fibrosis (Pirfenidone or Nintedanib)
symptoms chronic HP ( HP II)
progressive breathlessness and cough
Signs: may be crackles, clubbing is unusual
tests and what show for chronic HP?
CxR pulmonary fibrosis - most commonly in the upper zones
PFTs: restrictive defect (low FEV1 & FVC, high or normal ratio, low gas transfer - TLCO)
lung biopsy if in doubt.
treatment chronic HP?
remove antigen exposure, oral steroids if breathless or low gas transfer.
d: Idiopathic pulmonary fibrosis
chronic scarring lung disease characterized by a progressive and irreversible decline in lung function
aetiology of IPF?
imbalance of fibrotic repair system
related to gastric reflux
is IPf more/less common in smokers/
more
name some secondary causes of pulmonary fibrosis
rheumatoid, SLE, systemic sclerosis, asbestos
- drugs - amiodarone, busulphan, bleomycin, penicillamine, nitrofurantoin, methotrexate
symptoms IPF
progressive breathlessness (several years), dry cough
examination findings IPF?
clubbing, bilateral fine inspiratory crackles
restrictive defect on PFT’s - reduced FEV1 and FVC with normal or raised FEV1/FVC ratio, reduced lung volumes, low gas transfer
CXR- bilateral infiltrates
CT- reticulonodular fibrotic shadowing, worse at lung bases, honeycombing
pathology of IPF?
Usual Interstitial Pneumonia pattern (UIP) heterogenous fibrosis in alveolar walls with fibroblastic foci and destruction of architecture causing honeycombing. Inflammation is minimal
treatment of IPF III
Treatment is frustrating.
Steroids and immunosuppressants do not change course of disease
New antifibrotic drugs have emerged in recent years – PIRFENIDONE and NINTEDANIB – only therapies to have shown in randomised controlled trials to slow down disease progression BUT very expensive, and many side-effects.
Antifibrotic therapy does not reverse fibrosis, merely slows progression
Oxygen if hypoxic.
Lung transplantation in young patients
what is coal workers pneumonconiosis?
Complicated pneumoconiosis - progressive massive fibrosis restrictive pattern with breathlessness.
Chronic bronchitis (coal dust + smoking).
d; silicosis and how it presents
Simple pneumoconiosis – few symptoms;
chest X-ray abnormality (egg-shell calcification of hilar nodes).
Chronic silicosis - restrictive pattern, pulmonary fibrosis.
pleural diseases caused by asbestos and how they present
1- Benign pleural plaques - asymptomatic
2- Acute asbestos pleuritis - fever, pain, bloody pleural effusion
3- Pleural Effusion and Diffuse pleural thickening - restrictive impairment
4- Malignant Mesothelioma - incurable pleural cancer. Presents with chest pain and pleural effusion. No available treatment - fatal within two years.
name 2 other types of disease asbestos can cause?
Pulmonary Fibrosis - “Asbestosis” - heavy prolonged exposure. Diffuse pulmonary fibrosis and restrictive defect. Asbestos bodies in sputum. Asbestos fibres in lung biopsy.
Bronchial carcinoma - asbestos multiplies risk in smokers
