pulmoary fibrosis Flashcards
bi-basal fine crepitations on auscultation accompanied with hypoxia
pf
on CT
High resolution CT Chest: Reticulonodular shadowing and Honeycomb lung
on x ray
Chest X-ray: Bilateral lower zone reticulo-nodular shadowing
A 64-year-old man presents to his general practitioner with a 3-month history of progressively worsening shortness of breath that is worse on exertion and a non-productive cough.
Vital signs are stable. Spirometry reveals a forced expiratory volume in one second (FEV1) of 2.2 L, a forced vital capacity (FVC) of 2.5 L and FEV1/FVC of 88%
Which of the following is the most likely diagnosis?
restrictive as over 70%
idiopathic pulmonary fibrosis
restrictive spirometry due to the reduced DLCO, which happens as a result of the increased diffusion distance across the thickened, fibrotic alveoli - a process which would not occur in motor neurone disease or obesity hypoventilation syndrome
An 81-year-old man is reviewed on the acute medical ward. He presented due to a gradual increase in the size of his legs over the last 2 months. This is associated with worsening abdominal pain, specifically in his right upper quadrant. He was diagnosed with idiopathic pulmonary fibrosis 5 years ago. On examination he is cyanotic and has a JVP of 5cm above the sternal notch. On auscultation he has fine inspiratory crepitations and a pansystolic murmur heard loudest at the lower left sternal edge. He has 2cm hepatomegaly and bilateral pedal oedema up to his thighs. His chest X-ray demonstrates stable reticulo-nodular changes compared to his previous. His electrocardiogram (ECG) demonstrates sinus rhythm with right axis deviation. Which of the following is the next best investigation?
Echocardiogram
This man has evidence of cor pulmonale due to his chronic lung disease. An echocardiogram will be able to assess the extent of the damage to his ventricles and his valves. The murmur is suggestive of tricuspid regurgitation and the ECG changes are suggestive of right ventricular hypertrophy.
complications
Type 2 respiratory failure
Increased risk of lung cancer
Cor pulmonale
50% mortality in 5 years
what is cor pulmonale
abnormal enlargement of the right side of the heart as a result of disease of the lungs or the pulmonary blood vessels.
A 39-year-old man presents to the emergency department with a 6-month history of worsening shortness of breath on exertion, associated with a non-productive cough. He has no past medical history. He smokes 20 cigarettes a day and works on a farm. On examination, his respiratory rate is 33, oxygen saturations 91% on room air, heart rate 99, temperature 36.5, blood pressure 110/79mmHg. Auscultation of his chest reveals scattered inspiratory crepitations but no wheeze. Chest X-ray shows bilateral upper lobe reticulo-nodular shadowing. Spirometry was requested and reveals a restrictive pattern. Acid-fast bacilli in three sputum samples were negative. Which of the following is the next best investigation?
Serum precipitins for Aspergillus and mould antigens
The diagnosis here is extrinsic allergic alveolitis due to fungal spores, also known as “Farmer’s lung”. This can present acutely with fever, myalgia, dry cough and dyspnoea with bilateral airspace opacification suggestive of a pneumonitis on chest X-ray. It can also present with chronic fibrotic changes, which tend to have a predominance for the upper lobes. Serum precipitins are carried out to confirm exposure to fungal antigens.
what commonly causes pulmonary fibrosis in the upper lobes
Ankylosing spondylitis has a predominance for the upper lobes when it causes pulmonary fibrosis. The history of poorly controlled back pain in a young man with upper lobe fibrosis points to ankylosing spondylitis as the most likely diagnosis. Other causes of pulmonary fibrosis with a predominance for the upper lobes include tuberculosis, extrinsic allergic alveolitis, radiotherapy and sarcoidosis.
