Respiratory: IPF Flashcards
Describe what is meant by interstitial lung disease
Interstitial lung disease includes many conditions that cause inflammation and fibrosis of the lung parenchyma.
Describe the symptoms of IPF [3]
- Dyspnoea
- Non-productive cough
- Fatigue
Describe the signs of IPF [2]
Bibasal fine end-inspiratory crepitations
Clubbing
TOM TIP: Remember clubbing and bibasal fine inspiratory crackles in idiopathic pulmonary fibrosis. These patients are ideal for OSCEs as they are stable and have good signs.
Describe what imaging results would indicate IPF? [2]
CXR:
- basilar, peripheral, bilateral, asymmetrical, reticular opacities (shadowing of the lung peripheries)
High-resolution CT scan (HRCT) of the thorax - definitive imaging
- “ground glass” in early stages
- honeycombing in later stages
Describe where the fibrosis is in this CXR [1]
Pulmonary fibrosis causes reticular (net-like) shadowing of the lung peripheries which is typically more prominent towards the lung bases
Describe the spirometry results seen in IPF
Display a restrictive pattern:
- FEV1: decreased
- FVC decreased
(FEV1 and FVC are equally reduced)
- FEV1:FVC ratio greater than 70%
Which antibodies are present in 30% of patients in IPF? [1]
Which other compound might be found? [1]
Anti-nuclear antibodies - ANA
Rheumatoid factor is found in 10%
What does this CT depict in IPF? [1]
Honeycombing (advanced IPF)
What would be the next step when investigating IPF if history, clinical evaluation, and high-resolution CT findings do not support a clear diagnosis of IPF? [1]
Surgical lung biopsy (via VAT): fibrosis of varying ages; areas of normal lung next to areas of honeycombing
Samples from two distinct areas are required
How do you manage an acute axacerbation of IPF? [2]
- High dose oral prednisolone
- Condiser: cytotoxic therapy
BMJ BP
How do you manage a patient not currently experiencing an acute exacerbation of IPF? [3]
-
Pirfenidone: anti-fibronolytic therapy
OR
Nintedanib - Smoking cessation; pulmonary rehab & oxygen
- Consider: PPI if suffering from GORD symptoms: lomeprazole
- Patients experiencing progressive deterioration: lung transplant
BMJ BP
Explain the MoA of nintedanib? [1]
Nintedanib reduces fibrosis and inflammation by inhibiting tyrosine kinase
Name 4 therapeutic drugs that are a risk factor for causing PF [4]
Amiodarone (also causes grey/blue skin)
Cyclophosphamide
Methotrexate
Nitrofurantoin
Name 5 diseases that PF can occur secondary to [5]
Alpha-1 antitrypsin deficiency
Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Systemic sclerosis
Sarcoidosis
What is the prognosis of IPF? [1]
2-5 years life expectancy from diagnosis.
Which pathologies cause lung fibrosis in the upper [5] compared to lower zones [4]
Important to be able to ditinguish
Upper zones:
- hypersensitivity pneumonitis (also known as extrinsic allergic alveolitis)
- sarcoidosis
- TB
- Radiation-induced PF (post-breast / lung cancer; 12 months after)
- Coal workers penuomoconiosis
Lower zone:
- IPF
- Connective tissue disorders: e.g. SLE
- Drug induced: amiodarone, bleomycin, methotrexate
- Asbestosis
What is an acronym for causes of upper zone fibrosis? [5]
C - Coal worker’s pneumoconiosis
H - Histiocytosis/ hypersensitivity pneumonitis
A - Ankylosing spondylitis
R - Radiation
T - Tuberculosis
S - Silicosis/sarcoidosis
What is an acronym for causes of lower zone fibrosis? [4]
Lower (ACID)
- Asbestosis
- Connective tissue disorders (excluding Ank Spon eg. SLE)
- Idiopathic
- Drug induced (eg. Amiodarone, Bleomycin, Methotrexate)
Describe what is known as hypersensitivity pneumonitis [2]
Type III and type IV hypersensitivity reaction to an environmental allergen
Inhalation of allergens in patients sensitised to that allergen causes an immune response, leading to inflammation and damage to the lung tissue.
Name two drugs that can cause pulmonary fibrosis? [2]
Bleomycin & azathioprine
Which of the following medications could be responsible for causing the gentleman’s pulmonary fibrosis?
Aspirin
Ramipril
Bleomycin
Spironolactone
Simvastatin
Bleomycin
wWhich drug is a risk factor for causing pulmonary fibrosis? [1]
Methotrexate
What is a pneumonic for remembering causes of pulmonary fibrosis? [5]
CHARTS
Coal-worker pneumoconiosis
Histiocytosis/hypersensitivity pneumonitis
Ankylosing spondylitis / RA - Methotrexate use
Radiation
Tuberculosis
Silicosis/sarcoidosis
Asbesosis and Coal workers’ pneumoconiosis both cause interstitial lung fibrosis.
Where do each of them cause fibrosis in the lungs? [2]
Asbestos:
- lower lobes: in the roof and falls down
Silicosis and Coal workers’ pneumoconiosis:
- upper lobes drifts up from the ground to up
