pulmonary fibrosis and gradual onset breathlessness dr. tim sutherland consultant with a specialist...
TRANSCRIPT
Pulmonary Fibrosis and Gradual Onset Breathlessness
Dr. Tim SutherlandConsultant with a specialist interest in ILD
• How do patients with pulmonary fibrosis present?• When do I refer?• What are the common causes of pulmonary fibrosis?• How do we manage patients with pulmonary fibrosis?
Overview – through the three concerns of my friend
• Majority have progressive breathlessness over a period of time
• Other differentials include cardiac failure, COPD, obesity, deconditioning
• Associated medical conditions• Drugs (www.pneumotox.com)• Occupation• Environment• Smoking• Family history
Breathlessness and an approach to pulmonary fibrosis
• 81 year old gentleman• History of breathlessness and weight loss • Past medical history of vascular disease• Previous smoker• Crackles on auscultation and was clubbed• Spirometry shows a FVC of 86%
Recent cases
• How to interpret spirometry
First concern
• How to interpret spirometry
• Confirm the quality of the test• As a general rule 80-120% predicated is normal• Caution - treat generated reports with caution,
especially if they say the unexpected
First concern
• Much more common than restriction• Caused by a process that obstructs flow – generally in
larger airways• Typically causes reductions in FEV1 and FEV1/FVC
ratio• Causes of obstructive defects:
COPDAsthmaBronchiectasis/Cystic Fibrosis
Obstruction
• Restrictive defects are generally rare, esp in fibrosis• Caused by a process that reduces all lung volumes
- needs to be confirmed by a PFT lab• Causes:
(Intrinsic) - Interstitial lung diseases
(Extrinsic) - Kyphoscoliosis
- Ankylosing spondylitis
- Muscular dystrophies
Restriction
• Interpretation can only be:- Normal - Obstructive - Restrictive (rare)- Undetermined
Interpretation
• 47 year old gentleman• History of breathlessness over a year• No past medical history• Worked with aluminium, iron and steel• Previous smoker• Crackles on auscultation and was clubbed• Spirometry shows a FVC of 98%
Alternative presentation
• What do I do with this?
Second concern
• If in doubt, refer
Second concern
What causes pulmonary fibrosis?
Classification of Interstitial Lung Disease
Interstitial Lung Disease
OthersGranulomatous Disease
Idiopathic Interstitial Pneumonias
ILD ofknown cause
Chronic fibrosing
Smokingrelated
Acute/subacute
DrugsCTD
RA etc
IPFNSIP
RBILDDIP
COPAIP
LAMLCH etc
Classification of Interstitial Lung Disease
Interstitial Lung Disease
OthersGranulomatous Disease
Idiopathic Interstitial Pneumonias
ILD ofknown cause
Chronic fibrosing
Smokingrelated
Acute/subacute
DrugsCTD
RA etc
IPFNSIP
RBILDDIP
COPAIP
LAMLCH etc
• One of the most frequent diagnoses – approximately 30 cases per 100,000
• Used to be called CFA• No known cause• Tends to be older people, men more than women• No cure but there is treatment
Idiopathic Pulmonary Fibrosis
• Lung cancer survival • IPF survival
Thank god it’s not lung cancer Doctor!
• Lung cancer survival • IPF survival
Thank god it’s not lung cancer Doctor!
• Lung cancer survival • IPF survival
Thank god it’s not lung cancer Doctor!
• Lung cancer survival • IPF survival
• Main management is: - symptom control- oxygen assessment- pulmonary rehabilitation- planning for the future/managing
expectations- transplant?- newer medication
Thank god it’s not lung cancer Doctor!
• Antifibrotic agent – currently a red drug• Up to 9 capsules a day• Significant side effects – liver, sun-burn, GI• Requires LFT monitoring• We currently have about 110 patients on this drug
Pirfenidone
• Tyrosine kinase inhibitor – currently named patient only• 2 capsules a day• Fewer side effects• Requires LFT monitoring
Nintedanib
• If in doubt, call us or the specialist nurses• Control symptoms, check oxygen and discuss patient
expectations• Initiate future planning when able to
Third concern – how do I co-manage these patients?
• www.blf.org.uk• www.actionpulmonaryfibrosis.org
Any questions?