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Idiopathic Pulmonary
Fibrosis (IPF)Dr Helen Parfrey
Papworth Hospital, Cambridge
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IPF
What is IPF ?
What causes pulmonary fibrosis ?
How do I know if I have IPF ?
How is it treated ?
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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
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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”
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Fibrotic “Honeycomb” Lung
Normal Lung
Fibrotic or Honeycomb Lung
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IPF commonest form of pulmonary fibrosis
IPF
Autoimmune
disease
NSIP
Sarcoid
Drugs
Hypersensitivity
pneumonitisOccupati
on related
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Cause of IPF is unknown
Alveoli lining cells
Inhaled damaging particles
Repair process
es
Normal lung
tissue
Scar tissueLung
Fibrosis
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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
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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)
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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
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Establishing Diagnosis
Clinical assessment Evaluate symptoms Occupational exposures Medications Family history
Examination Oxygen saturation Clubbing of finger nails Listen to chest for
“crackles”
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Investigations
Blood tests
Lung Function Tests Forced Vital Capacity
(FVC) DLco or TLco (gas
transfer)
Walk test 6 minute walk test Shuttle walk test
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Chest X-ray
Normal IPF
Lung HeartLungs are reduced in size and have increased reticular markings
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Chest CT Scan
Normal IPF
Lung
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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
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Usual Interstitial Pneumonia (UIP)
Normal Lung UIP
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Establish DiagnosisClinical
• Symptoms• Smoking history• Exposures• Features of CTD• Examination
Investigations
• CXR• CT Thorax
• Blood tests
• Lung Function
Pathology
• Bronchoalveolar lavage
• Surgical lung biopsy
Multi-Disciplinary Team (MDT)
Discussion
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IPF Progressive Disease
Bjoraker et al AJRCCM 1998; 157: 199-203
Median survival is 3 years from diagnosis
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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
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What treatment options ?
Supportive• B
reathlessness management
• Oxygen
Symptoms• C
ough
Disease specific• M
edication
• Lung transplant
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Supportive Treatments
Smoking cessation
Breathlessness management Pacing, hand held fan Specialist clinic Medicines
Pulmonary Rehab and exercise Improves strength and walk distance
Palliative Care Services
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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
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Treating Cough
Cough suppression techniques –physiotherapy
Clearing phlegm Mucolytic agents such as mucodyne, N-
acetylcysteine (NAC)
Treat gastro-oesophageal reflux disease
Cough suppressant medications
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Preventing chest infections
Vaccination Annual flu vaccine Pneumonia vaccine
Prompt treatment of infections with antibiotics
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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
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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
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Questions