Resp - Idiopathic Pulmonary Fibrosis, Mesothelioma Flashcards
Idiopathic Pulmonary Fibrosis (IPF) - what is it?
Chronic lung condition characterised Progressive pulmonary fibrosis with no clear cause
Whilst there are many causes of lung fibrosis (e.g. medications, connective tissue disease, asbestos), term IPF reserved when no underlying cause exists
Fibrosis involves the replacement of the normal elastic and functional lung tissue with scar tissue that is stiff and does not function effectively
IPF - how does the disease present?
Presents with insidious onset of SoB and dry cough over > 3 months
IPF - what age does it commonly affect?
IPF is typically seen in patients aged 50-70 years
IPF - what are the classical clinical signs you would see on examination?
Finger clubbing
Bibasal fine inspiratory crackles
Other features you might see are:
- progressive exertional dyspnoea
- dry cough
IPF - how do you diagnose?
- Spirometry*: classically a restrictive picture (FEV1 normal/decreased, FVC signif decreased, FEV1/FVC increased)
- Imaging*: bilateral interstitial shadowing (typically small, irregular, peripheral opacities - ‘ground-glass’ - later progressing to ‘honeycombing’) may be seen on a chest x-ray but high-resolution CT scanning is the investigation of choice and required to make a diagnosis of IPF
Image shows X-Ray and CT from same patient - CT demonstrates honeycombing and traction bronchiectasis
IPF - prognosis?
Poor
Life expectancy 2-5 years from diagnosis
IPF - management?
Medications that can slow progression of disease:
Pirfenidone - antifibrotic and anti-inflammatory
Nintedanib - monoclonal antibody targeting tyrosine kinase
Pulmonary rehabilitation
Supplementary oxygen
Eventually a lung transplant
Mesothelioma - what is it?
Mesothelioma is a cancer of the mesothelial layer of the pleural cavity that is strongly associated with asbestos exposure
In a small percentage of cases, other mesothelial layers such as those in the abdomen may be affected
Malignancy of mesothelial cells of pleura
Mesothelioma - what are the clinical features?
Dyspnoea, weight loss, chest wall pain
Clubbing
30% present as painless pleural effusion
Breathlessness secondary to pleural effusions
History of asbestos exposure in 85-90%, latent period of 30-40 years
Mesothelioma - where does it metastasise to and which lung is affected more?
Metastases to contralateral lung and peritoneum
Right lung affected more often than left
Mesothelioma - investigations?
Gold Standard to confirm diagnosis - Thoracoscopic biopsy
CXR - pleural effusion or pleural thickening
Next step normally a pleural CT
If pleural effusion - fluid sent for MC&S, biochemistry and cytology
Local anaesthetic thoracoscopy
If an area of pleural nodularity is seen on CT then an image-guided pleural biopsy may be used
Mesothelioma - management?
Symptomatic
Chemotherapy
Surgery if operable
Prognosis poor, median survival 12 months
