Fibrotic Lung Disease Flashcards
What is fibrotic lung disease?
A restrictive lung disease caused by fibrosis to the lungs making breathing very difficult.
Name diseases associated with upper zone fibrotic lung disease.
CHARTS !!!
C- Coal workers pneumoconisosis
H - Histiocytosis/hypersensitivity penumonitis
A - Ankylosing spondylitis
R - Radiation
T - TB
S - Silicosis/sarcoidosis
What is the most common condition in lower zone fibrotic lung disease?
ACIDS
Idiopathic pulmonary fibrosis (IPF)
What are the causes of lower zone pulomnary fibrosis?
AAAI
A - Asbestos
A - Amioderone
A - Autoimmune conditions (SLE, RA)
I - Idiopathic pulmonary fibrosis
Define idiopathic pulmonary fibrosis (IPF).
A restrictive chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs - IPF occurs when there is NO underlying cause.
What is the typical demographic for idiopathic pulmonary fibrosis?
Common in ages 50-70, predominantly MALE.
What are the symptoms of idiopathic pulmonary fibrosis?
- Progressive dyspnoea on exercise
- Dry cough
What are the signs of idiopathic pulmonary fibrosis?
Auscultation: Bi-basal fine end-inspiratory crepitations
Clubbing.
What is the spirometry pattern in idiopathic pulmonary fibrosis?
Classically a restrictive pattern.
What are the typical spirometry values in idiopathic pulmonary fibrosis?
- FEV1 - normal/decreased
- FVC - decreased
- FEV1/FVC - increased (above 0.7)
- TLCO (imapired gas exchange) - REDUCED
What does TLCO measure?
Transfer factor of lung for carbon monoxide, indicating diffusion of gas across the alveolar blood membrane.
What imaging techniques are used for diagnosing fibrotic lung disease?
- CXR for investigation
- High-resolution CT for diagnosis
What are positive findings on imaging (CXR or CT) for idiopathic pulmonary fibrosis?
- Bilateral interstitial shadowing
- Ground glass
- Honeycombing
What blood tests are relevant for idiopathic pulmonary fibrosis?
- ANA positive in 30%
- Rheumatoid factor + in 10%
What is the management strategy for idiopathic pulmonary fibrosis?
- Pulmonary rehabilitation
- Drugs: Pirfenidone (slows down disease progression)
- Long-term: Supplementary O2 + lung transplant
What is the prognosis for idiopathic pulmonary fibrosis?
Poor life expectancy = 3-4 years.