Idiopathic pulmonary fibrosis Flashcards
1
Q
Idiopathic pulmonary fibrosis definition
A
- chronic, life-threatening disease
- formation of scar tissue in lungs
- increased shortness of breath
2
Q
Idiopathic pulmonary fibrosis risk factors
A
- family history
- smoking
- increasing age (> 50)
- more common in males
3
Q
Idiopathic pulmonary fibrosis pathophysiology
A
- unknown, insult may damage alveolar epithelium, endothelium and basement membrane
- inflammatory cells damage lung
- repair process dysregulation
4
Q
Idiopathic pulmonary fibrosis signs and symptoms
A
- progressive exertional dyspnoea
- bibasal fine end-inspiratory crackles
- dry cough
- clubbing
5
Q
Idiopathic pulmonary fibrosis investigations
A
- restrictive picture on spirometry
- impaired gas exchange
- ground glass appearance on CT
- honeycombing pattern
6
Q
Idiopathic pulmonary fibrosis management
A
- pulmonary rehabilitation
- pirfenidone and nintedanib may be effective
- lung transplant in long term
7
Q
Fibrosis in upper zones
A
“CHARTS”
* Coal workers’ pneumonitis
* Hypersensitivity
* Ankylosing spondylosis
* Radiation
* TB
* Silicosis – egg shell calcification
* Sarcoidosis
8
Q
Fibrosis in lower zones
A
- Idiopathic pulmonary fibrosis
- Connective tissue disorders, eg SLE
- Drug induced (amiodarone,
bleomycin, methotrexate) - Asbestosis
