Respiratory: fibrosis Flashcards
1
Q
What are the clinical features of idiopathic pulmonary fibrosis?
A
- progressive exertional dyspnoea
- bibasal fine end-inspiratory crepitations on auscultation
- dry cough
- clubbing
2
Q
How is IPF diagnosed?
A
- spirometry: restrictive picture (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased)
- impaired gas exchange: reduced transfer factor (TLCO)
- imaging: bilateral interstitial shadowing (typically small, irregular, peripheral opacities - ‘ground-glass’ - later progressing to ‘honeycombing’) may be seen on CXR but HRCT is the investigation of choice and required to make a diagnosis of IPF
- ANA positive in 30%, rheumatoid factor positive in 10% but this does not necessarily mean that the fibrosis is secondary to a connective tissue disease. Titres are usually low
3
Q
What is the management for IPF?
A
- pulmonary rehabilitation
- very few medications have been shown to give any benefit in IPF
- some evidence that pirfenidone (an antifibrotic agent) may be useful in selected patients
- many patients will require supplementary oxygen and eventually a lung transplant (life expectancy 3-4 years)
4
Q
What are the respiratory manifestations of RA?
A
- pulmonary fibrosis
- pleural effusion
- pulmonary nodules
- bronchiolitis obliterans
- complications of drug therapy e.g. methotrexate pneumonitis
- pleurisy
- Caplan’s syndrome - massive fibrotic nodules with occupational coal dust exposure
- infection (possibly atypical) secondary to immunosuppression
5
Q
What is sarcoidosis?
A
- multisystem disorder (unknown aetiology) characterised by non-caseating granulomas.
- It is more common in young adults and in people of African descent
6
Q
Which drugs can cause pulmonary fibrosis?
A
- Cytotoxic drugs e.g. busuplhan, bleomycin, methotrexate, cyclophosphamide,rituximab.
- Antibiotics e.g. nitrofurantoin
- Cardiac drugs e.g. hydralazine, amiodarone, tocainide
- Opioids e.g. heroin abuse
