7 steps How to prevent Thalassemia : Dr Sharda Jain & Vandana Gupta
What is Pulmonary Fibrosis?
1. What is pulmonary fibrosis
and why it is difficult to
diagnose (and how we do it)?
Dr Katherine Spinks
Consultant Respiratory Physician
Southern Health (Lymington, Hythe and Romsey
Hospitals) and University Hospital of Southampton
NHS Trusts
2. So, what is pulmonary fibrosis?
• Term for a group of conditions, all of which
cause the lungs to become stiff, small and less
good at transferring oxygen from the air to the
body because of the formation of excess fibrous
/ scar tissue.
• Many different causes
• Treatment depends on the cause.
• Some conditions are progressive.
5. Some commoner types of pulmonary fibrosis
and causes
• Idiopathic Pulmonary
Fibrosis
– Genetics?
– Exposure to cigarette
smoke and/or other inhaled
allergens or particles?
• Sarcoid – largely unknown
• Smoking related
(RBILD, DIP)
• Drug induced eg
amiodarone, nitrofurantoin
• Unclassifiable
• Hypersensitivity
pneumonitis (HP) –
exposure to inhaled allergens
eg birds
• Asbestosis – former
asbestos exposure >20
years ago
• Connective tissue
associated – part of
whole body auto-immune
condition eg Rheumatoid
arthritis, Sjogrens syndrome.
Characterised by inflammation.
6. BTS IPF registry 2016
• The average hospital receives ~700 new
respiratory referrals/ year, on average 37
are for suspected IPF (5%).
• 64% of IPF patients are aged over 70
• 46% have had symptoms for more than 2
years
• 66% are ex-smokers
• 20% have heart disease
7. Symptoms of lung disease
• Breathlessness
• Dry cough
• Wheeze
• Sputum
• Chest pain
• Common in ILD (and
heart disease, COPD,
asthma, GORD, obesity)
Uncommon in ILD –
suggests airways
disease, infection or
even lung cancer
Some people have more than one condition
8. In other words…
Pulmonary fibrosis is relatively rare, occurs
in older people and has non-specific
symptoms that might be due to other kinds
of commoner lung or heart disease
Patients often have symptoms for
some time before a diagnosis is made.
9. How do we diagnose it ?
Take a history
• Duration of and change in
symptoms
• Smoking and occupational
history (farming, dusts)
• Exposure history (pets,
birds, asbestos)
• Other medical history
• Medication history
• Family history
Examine the patient
The lungs:
• Crackles?
• Squeaks and squawks?
• Nothing?
The rest of the body:
Finger clubbing?
Arthritis?
Skin lesions?
Swollen lymph nodes?
10. Investigations
• Breathing tests (PFTS)
Patients with ILD tend to
have small lungs that
cannot transfer
oxygen from
the air to the
bloodstream.
Also used for
monitoring
disease progression /
response to treatment.
• Blood tests
– Can look for
underlying conditions
that are associated
with lung fibrosis eg
autoimmune disease,
signs of infection,
allergy to specific
airborne irritants and
clues to other causes
of breathlessness eg
heart disease.
11. How do we do it 2 ?
Special tests :
– CXR and CT scan
– Lung Biopsy?
15. How do we do it (3)
• Amalgamate all the information together to
try and make a diagnosis and treatment
plan.
• Referral to a specialist centre often
needed to access subspecialists and
certain treatments eg anti-fibrotic therapy.
16. MDT (multi-disciplinary meeting)
• Meeting with chest physicians,
radiologists, (surgeons), pathologists,
nurse specialists.
• Discuss the history, clinical findings and
test results.
• Either decide that we know the diagnosis
or that we need more information.
• If we know the diagnosis recommend a
management / treatment plan.
17. Summary
• The ILDs are not very common
• They have non-specific symptoms
• A high index of suspicion is needed
• Special tests are required to diagnose
• Final diagnosis often needs multiple
doctors in a specialty centre.
• It can be confusing!
