This document discusses idiopathic pulmonary fibrosis (IPF). IPF is a lung disease where scar tissue builds up in the lungs, impairing gas exchange. The cause is unknown but it usually affects older males. Symptoms include cough and shortness of breath. Diagnosis involves clinical assessment, imaging like CT scans, and sometimes biopsies. While there is no cure, treatments can help slow progression and include oxygen therapy, pulmonary rehabilitation, and medications like pirfenidone. The long-term prognosis is generally poor with median survival of 3 years from diagnosis.

1. Idiopathic
Pulmonary Fibrosis
(IPF)
Dr Helen Parfrey
Papworth Hospital, Cambridge

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”

5. Fibrotic “Honeycomb” Lung
Normal Lung Fibrotic or 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

13. Chest X-ray
Normal IPF
Lung Heart
Lungs are reduced in size and have
increased reticular markings

14. Chest CT Scan
Normal IPF
Lung

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

16. Usual Interstitial Pneumonia (UIP)
Normal Lung UIP

17. Establish Diagnosis
Clinical
• Symptoms
• Smoking history
• Exposures
• Features of CTD
• Examination
Investigations
• CXR
• CTThorax
• Blood tests
• Lung Function
Pathology
• Bronchoalveolar
lavage
• Surgical lung
biopsy
Multi-DisciplinaryTeam (MDT)
Discussion

18. IPF Progressive Disease
Bjoraker et al AJRCCM 1998; 157: 199-203
Median survival is 3 years from diagnosis

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

20. What treatment options ?
Supportive
• Breathlessness management
• Oxygen
Symptoms
• Cough
Disease specific
• Medication
• Lung transplant

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

23. Treating Cough
 Cough suppression techniques –physiotherapy
 Clearing phlegm
 Mucolytic agents such as mucodyne, N-acetylcysteine
(NAC)
 Treat gastro-oesophageal reflux disease
 Cough suppressant medications

24. Preventing chest infections
 Vaccination
 Annual flu vaccine
 Pneumonia vaccine
 Prompt treatment of infections with antibiotics

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

28. Questions