2. IPF
What is IPF ?
What causes pulmonary fibrosis ?
How do I know if I have IPF ?
How is it treated ?
3. Lung Structure
The airways branch
within the lung
End in air sacs or alveoli
Sites where exchange
oxygen breathe in with
carbon dioxide breathe
out
4. What is pulmonary fibrosis?
Scar tissue or collagen
builds up around the alveoli
- this process is termed
FIBROSIS
Impairs gas exchange in the
lung
Accumulation of scar tissue
over time permanently
destroys lung structure
Leads to “honeycomb lung”
6. IPF commonest form of
pulmonary fibrosis
IPFAutoimmune
disease
NSIP
SarcoidDrugs
Hypersensitivity
pneumonitisOccupation
related
7. Cause of IPF is unknown
Alveoli lining cells
Inhaled damaging
particles
Repair
processes
Normal
lung tissue
Scar tissue
Lung Fibrosis
8. Who gets IPF ?
Males > Females (2:1)
Increasing age (>60 yrs)
Estimated 15000 people with IPF in UK
5000 new cases IPF per year in UK
Incidence increasing in worldwide
Growing health concern
9. Risk factors for developing IPF
Smoking (2.8 fold increased risk)
Occupational exposures
Hard woods, metals, asbestos
Family history of pulmonary fibrosis
Possibly gastro-oesophageal reflux disease
(GORD)
10. What are the symptoms of IPF?
Symptoms
Cough
Dry tickle
Productive of sputum
Short of breath with
activities
Limitations
Symptoms common to
many lung diseases
Delay in diagnosis
11. Establishing Diagnosis
Clinical assessment
Evaluate symptoms
Occupational exposures
Medications
Family history
Examination
Oxygen saturation
Clubbing of finger nails
Listen to chest for “crackles”
12. Investigations
Blood tests
Lung Function Tests
ForcedVital Capacity (FVC)
DLco orTLco (gas transfer)
Walk test
6 minute walk test
Shuttle walk test
15. If diagnosis is uncertain…..
Some circumstances the CT scan has unusual
features which are not typical for IPF
May need a bronchoscopy
May need a surgical lung biopsy
19. How to treat IPF
No curative treatment other than lung transplant
Aim of treatment is to slow rate of progression of
IPF
Rate of progression is very variable
Monitor change in lung function (FVC)
Not all patients with IPF may treatment
21. SupportiveTreatments
Smoking cessation
Breathlessness management
Pacing, hand held fan
Specialist clinic
Medicines
Pulmonary Rehab and exercise
Improves strength and walk distance
Palliative Care Services
22. Oxygen therapy
Not for everyone with IPF
People who are limited by low blood oxygen
walking outside / gardening
around the house
at night
all or most of the time
Different types of oxygen
Long term oxygen therapy (LTOT)
Oxygen for exercise (ambulatory)
Short burst
24. Preventing chest infections
Vaccination
Annual flu vaccine
Pneumonia vaccine
Prompt treatment of infections with antibiotics
25.
26. Treatments for IPF
Prednisolone and azathioprine +/- N-acetylcysteine
Use of this has been questioned by interim report from the PANTHER
study in the USA
May wish to discuss this treatment with your doctor
Pirfenidone (Esbriet)
First licensed treatment for IPF in Europe
Recommended for mild to moderate IPF
Named patient programme in UK
Slow disease progression
Side effects – skin rash, GI symptoms, liver impairment
Discuss taking part in a clinical trial
Discuss lung transplantation
27. Conclusions
Number of challenges remain
Establishing the diagnosis can be difficult
Limited treatment options and accessibility
Poorly identified patient needs
Limited resources available for patients and families
Promoting awareness and education