Lung fibrosis Flashcards
sarcoidosis, pulmonary fibrosis, asbestosis, extrinsic allergic alveolitis (24 cards)
Give 5 conditions that cause predominately upper zone fibrosis
- hypersensitivity pneumonitis
- sarcoidosis
- ankylosing spondylitis
- radiation-induced pulmonary fibrosis
- tuberculosis
Give 4 causes of predominately lower zone fibrosis
- idiopathic pulmonary fibrosis
- most connective tissue disorders e.g. SLE
- drug-induced: amiodarone, bleomycin, methotrexate
- asbestosis
What is idiopathic pulmonary fibrosis
a chronic lung condition characterised by progressive fibrosis of the lungs with no apparent cause
Risk factors for idiopathic pulmonary fibrosis
- older age (60-70 years)
- male
- FHx
Features of idiopathic pulmonary fibrosis
- progressive exertional dyspnoea
- fine end-inspiratory fine bi-basal fine crackles on auscultation
- dry cough
- clubbing
What are the typical findings in spirometry for idiopathic pulmonary fibrosis?
Restrictive pattern:
* FEV1 normal or decreased
* FVC decreased
* FEV1/FVC increased
What is the typical imaging finding for idiopathic pulmonary fibrosis?
- Chest x-ray: peripheral, Bilateral, asymmetrical, reticular opacities (ground-glass)
- High-resolution CT chest (GS) - increased reticulation, honeycombing
What is the management for idiopathic pulmonary fibrosis?
- acute - admission, supportive care, prednisolone
- Antifibrotic agent: pirfenidone or nintedanib
- pulmonary rehabilitation
What is sarcoidosis?
A chronic multisystem disorder of unknown aetiology characterised by non-caseating granulomas
What are granulomas
inflammatory nodules full of macrophages
Describe the epidemiology of sarcoidosis
- 20-40 year old
- women
- black ethnic origin
What is the most commonly affected organ in sarcoidosis
lungs
How are the lungs affected by sarcoidosis
- patients may present with dry cough and gradual dyspnoea
- bilateral hilar lymphadenopathy
- pulmonary nodules
- pulmonary fibrosis
Skin manifestations of sarcoidosis
- erythema nodosum - tender, red nodules on shins
- lupus pernio - raised purple plaques on nose, cheeks
Systemic features of sarcoidosis
- fever
- fatigue
- weight loss
Ocular symptoms of sarcoidosis
- uveitis
- conjunctivitis
What electrolyte change may be seen in sarcoidosis
hypercalcaemia
What is Lofgren’s syndrome
acute form of sarcoidosis characterised by:
* bilateral hilar lymphadenopathy
* erythema nodosum
* polyarthralgia
What blood tests are done to investigate sarcoidosis
- serum angiotensin-converting enzyme (ACE) - raised
- calcium - raised
- ESR - raised
What imaging is involved in the diagnosis of sarcoidosis
- CXR - lymphadenopathy, interstitial infiltrates, fibrosis
- MRI - identify areas of sarcoidosis involvement
What would histology of sarcoidosis characteristically show
non-caseating granulomas with epithelioid cells
How is sarcoidosis managed
- asymptomatic - conservative
- symptomatic: 1st - steroids, 2nd - methotrexate
What is the prognosis for sarcoidosis
- spontaneously resolves in around 50% of patients with stage I/II disease
- can progress to pulmonary fibrosis in some patients
Give 3 factors associated with a poor prognosis for sarcoidosis
- insidious onset, symptoms > 6 months
- extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
- CXR: stage III-IV features
