Respiratory 1 Flashcards
what is cor pulmonale?
enlargement and failure of right side of the heart due to disease of lungs/pulmonary blood vessels - leads to oedema and raised JVP
describe what is happening in the lungs of a patient with extrinsic allergic alveolitis (hypersensitivity pneumonitis)
inhalation of allergens provokes a hypersensitivity reaction, with complement activation, granuloma formation and obliterative bronchiolitis.
give 2 causes of EAA (hypersensitivity pneumonitis)
Farmer's lung. Bird-fancier's lung - proteins in bird droppings. Malt-worker's lung. Bagassosis/Sugar worker's lung. humidifier fever. Mushroom workers. Cheese washer's lung. Wine maker's lung.
give 3 clinical features of EAA seen after exposure to the allergen
fevers, rigors, myalgia, dry cough, dyspnoea, crackles (no wheeze)
give 3 chronic features of EAA
increasing dyspnoea, weight loss, exertional dyspnoea, type I respiratory failure, cor pulmonale
what would been seen on CXR of a patient with EAA?
fibrosis/mottling of upper lobes and honeycomb lung
list some investigations that might be performed on a patient with EAA
bloods - neutrophilia, raised ESR
CXR.
lung function tests (reversible restrictive).
broncheoalveolar lavage.
how would you treat EAA in an acute and a chronic situation?
acute - remove allergen, give O2 + oral prednisolone.
chronic - avoid exposure (facemask), long-term steroids.
list 3 occupational lung diseases
EAA (e.g. Farmer's lung). Coal worker's pneumonconiosis. Silicosis. Asbestosis. Byssinosis. Berylliosis.
what causes the fibrosis seen in coal worker’s pneumoconiosis?
inhalation of coal dust particles - ingested by macrophages - these die and release their enzymes - fibrosis
what would a CXR show in coal worker’s pneumoconiosis?
round opacities in upper zone.
what causes progressive massive fibrosis? what are the features of this?
progression of coal worker’s pneumoconiosis.
progressive dyspnoea, fibrosis + eventual cor pulmonale.
give some examples of jobs at risk of silicosis
metal mining, stone quarrying, sand blasting, pottery/ceramic manufacture
what do investigations show in silicosis?
CXR - diffuse miliary/nodular pattern in upper and mid-zones + egg shell calcification of hilar nodes.
Spirometry - restrictive.
what disease are patients with silicosis at greater risk of?
TB
what are the clinical features of asbestosis?
progressive dyspnoea.
O/E - clubbing, fine end-inspiratory crackles, pleural plaques.
what two diseases are asbestosis patients at greater risk of?
bronchial adenocarcinoma and mesothelioma
in what industries might workers get byssinosis? and for berylliosis?
byssinosis - cotton mill workers.
berylliosis - beryllium-copper alloy used in aerospace industry, electronics, atomic reactors.
describe the pathogenesis of bronchiectasis
chronic infection of bronchi/bronchioles leads to inflamed, thickened and irreversibly damaged walls with permanent dilation.
mucociliary transport mechanism is impaired.
give 2 of the main organisms involved in bronchiectasis
H influenza, Strep pneumonia, Staph aureus, Pseudomonas aeruginosa
give 3 possible causes of bronchiectasis
congenital - CF.
post-infection - measles, pertussis, pneumonia, TB, HIV.
Other - bronchial obstruction (tumour, foreign body), allergic bronchopulmonary aspergillosis (ABPA), hypogammaglobulinaemia, rheumatoid arthritis, UC.
give 3 clinical features of bronchiectasis
persistent cough, copious purulent sputum, intermittent haemoptysis, finger clubbing, coarse inspiratory crepitations, wheeze
give 2 possible complications of bronchiectasis
pneumonia, pleural effusion, pneumothorax, haemoptysis, cerebral abscess, amyloidosis
name 3 investigations you would carry out in bronchiectasis and their results
*sputum culture.
*CT scan - shows the dilated airways.
CXR - cystic shadows, thickened bronchial walls.
spirometry - obstructive pattern.
broncoscopy - locate site of haemoptysis, exclude obstruction, obtain samples.
how would you manage a patient with bronchiectasis?
physiotherapy - postural drainage.
Abx - flucloxacillin for staph, amoxicillin for strep, tazocin for pseudomonas.
bronchodilators - salbutamol nebulisers.
Oral/inhaled corticosteroids.
what causes cystic fibrosis?
autosomal recessive mutation in the CF transmembrane conductase regulator gene on chromsome 7.
defective chloride secretion and increased sodium absorption over airway epithelium - produces very viscous and sticky mucous.
give 3 respiratory symptoms of CF
cough, wheeze, recurrent infections, bronchiectasis, pneumothorax, haemoptysis, respiratory failure, cor pulmonale
give 3 extrapulmonary features of CF
pancreatic insufficiency - DM, steatorrhoea.
intestinal obstruction, gallstones, cirrhosis, male infertility, osteoporosis, arthritis, vasculitis, sinusitis.
name 3 investigations you would carry out in CF and their results
sweat test - increased sodium and chloride secretion in sweat.
faecal elastase - screens for pancreatic dysfunction.
genetic screening for CF mutations.
CXR - hyperinflation, bronchiectasis.
how would CF be managed?
physiotherapy. Abx for exacerbations. mucolytics - DNase (dornase alfa). bronchodilators. fat soluble vit supplements. pancreatic enzyme replacement.
what is sarcoidosis? what genes is it associated with?
multisystem granulomatous disorder of unknown cause.
associated with HLA-DRB1 and DQB1 alleles.
what is seen on transbronchial biopsy in sarcoidosis?
infiltration of alveolar walls and interstitial spaces with mononuclear cells - later, granulomas
how does acute sarcoidosis present?
erythema nodosum ± polyarthralgia
give 3 pulmonary features of sarcoidosis?
dry cough, progressive dyspnoea, decreased exercise tolerance, chest pain.
give 3 extra-pulmonary features of sarcoidosis
lymphadenopathy, hepatomegaly, splenomegaly, uveitis, conjunctivitis, glaucoma, Bell’s palsy, neuropathy, meningitis, brainstem and spinal syndromes, space occupying lesions, erythema nodosum, cardiomyopathy, arrhythmias, hypercalcaemia, renal stones, pituitary dysfunction
what are the features of sarcoidosis on CXR?
bilateral hilar lymphadenopathy ± pulmonary infiltrates/fibrosis
list some differential diagnoses for bilateral hilar lymphadenopathy
sarcoidosis, infection (TB, mycoplasma), malignancies, silicosis, EAA
what investigations would you carry out, apart from CXR, in sarcoidosis, and what might they show?
SERUM ACE is raised.
lung function - restrictive pattern, reduced TLC, reduced FEV1/FVC ratio.
tissue biopsy - non-caseating granuloma.