Idiopathic Pulmonary

Fibrosis (IPF)Dr Helen Parfrey

Papworth Hospital, Cambridge

IPF

What is IPF ?

What causes pulmonary fibrosis ?

How do I know if I have IPF ?

How is it treated ?

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

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”

Fibrotic “Honeycomb” Lung

Normal Lung

Fibrotic or Honeycomb Lung

IPF commonest form of pulmonary fibrosis

IPF

Autoimmune

disease

NSIP

Sarcoid

Drugs

Hypersensitivity

pneumonitisOccupati

on related

Cause of IPF is unknown

Alveoli lining cells

Inhaled damaging particles

Repair process

es

Normal lung

tissue

Scar tissueLung

Fibrosis

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

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)

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

Establishing Diagnosis

Clinical assessment Evaluate symptoms Occupational exposures Medications Family history

Examination Oxygen saturation Clubbing of finger nails Listen to chest for

“crackles”

Investigations

Blood tests

Lung Function Tests Forced Vital Capacity

(FVC) DLco or TLco (gas

transfer)

Walk test 6 minute walk test Shuttle walk test

Chest X-ray

Normal IPF

Lung HeartLungs are reduced in size and have increased reticular markings

Chest CT Scan

Normal IPF

Lung

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

Usual Interstitial Pneumonia (UIP)

Normal Lung UIP

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

IPF Progressive Disease

Bjoraker et al AJRCCM 1998; 157: 199-203

Median survival is 3 years from diagnosis

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

What treatment options ?

Supportive• B

reathlessness management

• Oxygen

Symptoms• C

ough

Disease specific• M

edication

• Lung transplant

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

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

Treating Cough

Cough suppression techniques –physiotherapy

Clearing phlegm Mucolytic agents such as mucodyne, N-

acetylcysteine (NAC)

Treat gastro-oesophageal reflux disease

Cough suppressant medications

Preventing chest infections

Vaccination Annual flu vaccine Pneumonia vaccine

Prompt treatment of infections with antibiotics

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

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

Questions