1. health and fitness
2. disease

Idiopathic Pulmonary
Fibrosis
Diagnosis and Treatment
Dr Paul Beirne
Leeds Teaching Hospitals NHS Trust
Idiopathic Pulmonary Fibrosis
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How does IPF present?
How is IPF diagnosed?
Tests
 IPF versus ‘Non-IPF’ pulmonary fibrosis
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How is IPF managed?
Monitoring
 Active treatment
 Supportive treatment
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What is pulmonary fibrosis?
Pulmonary fibrosis can be the result of many different
lung diseases, including IPF
Symptoms of Pulmonary Fibrosis
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Shortness of breath on exercise
Dry cough
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None
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Signs of Pulmonary Fibrosis
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Digital clubbing 25-50%
On listening to the chest:
Fine late inspiratory crackles (‘Velcro’ crackles)
at lung bases
Accurate Diagnosis
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Accurate diagnosis requires taking a thorough history
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Occupational history
Drug history
Environmental history
Smoking history
Rheumatological history
High resolution CT (HRCT) scan of the chest
Blood tests
Lung function tests
Exercise test (6 minute walk test)
Photo by Joe Mabel
Interstitial Lung Disease
Multidisciplinary Team Discussion
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Respiratory physician
Radiologist
Pathologist
Cardiothoracic Surgeon
Rheumatologist
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Diagnosis of IPF confirmed?
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IPF or ‘Non-IPF’?
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Not all pulmonary fibrosis is IPF
Not all cases of IPF have the classical HRCT
appearances
If case not consistent with IPF then alternative
diagnoses, causes and treatments should be
considered
This may require further tests
Bronchoscopy, lavage, biopsy
 Surgical lung biopsy (VATS)
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IPF
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A distinctive type of chronic fibrosing interstitial
pneumonia of unknown cause limited to the lungs and
associated with a surgical biopsy showing Usual
Interstitial Pneumonia (UIP):
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architectural destruction by fibrosis, often with
honeycombing, with fibroblastic foci and patchy distribution
including some areas of normal lung and some areas of mild
to moderate inflammation
Usually aged over 50 (median age 70), insidious onset
SOB and dry cough
Male: female ratio 1.5 – 2:1
Crackles +/- digital clubbing
Can usually be diagnosed without a lung biopsy
IPF – Natural History
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Unpredictable:
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Gradual steady deterioration
Periods of stability possible – often prolonged
Periods of accelerated decline may occur
IPF is associated with an increased risk of lung cancer
which interacts with smoking
Median survival from diagnosis?
Information and Education
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Large amount of information available to
patients, particularly on the internet…
Information and Education
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Provision of clear and accurate information verbal and written – is essential
Patient leaflets
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British Lung Foundation literature
Nurse education
Smoking cessation advice and help
Access to advice and support (e.g. telephone
help line)
Information and Education:
the specialist nurse
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Support and information to patients
Point of contact
Liaison with other social and healthcare professionals
 Hospital team
 GPs
 Community matrons/nurses
 Social workers
 Palliative care teams
Follow-up
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Monitoring
Lung function tests at 6 to 12 month intervals
 Exercise testing e.g. 6 minute walk test
 Oxygen requirements (oxygen saturations, exercise
tolerance, symptoms)
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Treatment
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Pharmacological
NAC
 Perfenidone
 Steroids
 Azathioprine
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Non-pharmacological
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Best Supportive Care
Best Supportive Care
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Defined by WHO:
‘…an approach that improves the quality of life of
patients and their families facing the problems
associated with a life-threatening illness, through the
prevention and relief of suffering by means of early
identification and impeccable assessment of pain and
other problems, physical, psychosocial and spiritual’
Best Supportive Care
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Smoking cessation
Treatment of acid reflux
Treatment of cough
Pulmonary rehabilitation
Oxygen
Avoidance, dose reduction or withdrawal of therapy
that does significant harm without perceivable benefit
Early recognition of decline that may require input
from palliative care services
Open and honest communication
Acid reflux
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Gastro-oesophageal reflux disease (GORD) is
more common in IPF
May be asymptomatic
May be causing or aggravating any cough
Role in disease causation?
All symptomatic patients should be treated
Protein pump inhibitor
 Gastric motility agent
 Gaviscon Advance suspension for cough
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Cough
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?GORD
?coexisting chronic bronchitis
Cough suppressants
Pholcodine linctus
 Codeine
 Opiates
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Pulmonary Rehabilitation
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Courses mixing physical exercises, education,
psychological and social support
Usually run by nurses, physiotherapists,
psychologists, dieticians
Well established in COPD
Reduced breathlessness
 Improved quality of life
 Fewer hospital admissions
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Evidence for benefit in IPF is lacking, but BTS
Guidelines (2008) recommended IPF patients be
considered along same lines as COPD patients
Oxygen
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3 basic ways that oxygen is prescribed:
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Long-term oxygen therapy (LTOT)
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Short-burst oxygen
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For symptomatic relief of breathlessness
Ambulatory oxygen
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To correct dangerously low oxygen levels
To improve exercise tolerance in people mobile outside
the home
Prescribed via a Home Oxygen Order Form
(HOOF)
Long term oxygen therapy (LTOT)
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Via an oxygen concentrator for at least 15 hours
per day
For patients with PaO2 < 7.3 kPa on air at rest,
or < 8 kPa with evidence of pulmonary
hypertension
In IPF, high flow rates may be required
Oxygen concentrator for
LTOT
70 x 42 x 37 cm
Up to 5 lpm
May be required in tandem
for higher rates of oxygen
delivery
Short burst oxygen therapy
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10 to 20 minutes at a time, usually from a
cylinder
For relief of breathlessness associated with
hypoxia not requiring LTOT or ambulatory
oxygen
Cylinders for:
SBOT
Ambulatory oxygen
+/- Conserving
devices
Ambulatory oxygen
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For patients who desaturate on exercise to SaO2
< 90% and in whom reduced breathlessness or
improved exercise tolerance can be
demonstrated on supplemental oxygen
Requires an ambulatory oxygen assessment
Palliative Care
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GPs may seek guidance from the hospital on when to
involve the Palliative Care Team
Need for Palliative Care Team input is assessed in
clinic, and also through telephone consultations if a
patient is too ill to attend outpatients
IPF patients would benefit from closer links between
IPF team and community and hospital-based palliative
care teams
Palliative
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Opiates
Benzodiazepines
Transplant
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Refer if disease is advanced (gas transfer < 40%
predicted, or if evidence of a rapid decline)
Many IPF patients cannot be considered by the
transplant centres
Comorbidities
 Age
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Summary
Accurate diagnosis
Information and education
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Patient
Healthcare professionals
Treatment
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Pharmacological
Non-pharmacological
Follow-up
Transplant referral
Support
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Best Supportive Care