1. How is pulmonary fibrosis diagnosed and monitored?
Dr Paul Beirne

2. To discuss Who gets pulmonary fibrosis? Symptoms and signs Radiology
Lung function
Monitoring over time
Oxygen assessment
Complications of pulmonary fibrosis

3. Who gets pulmonary fibrosis?
Commonest form is Idiopathic Pulmonary Fibrosis (IPF)
Average age 65
Uncommon below age 50
More common in men
Other forms of pulmonary fibrosis
Depends on underlying diagnosis/cause

4. Symptoms
Cough
Breathlessness on exertion
None

5. Signs
Crackles
Digital clubbing

6. Digital clubbing

7. Initial tests (GP)
Chest x-ray
Spirometry

8. Respiratory Specialist
History
Medical history
Family history
Smoking history
Occupational history
Environmental exposures
Birds
Damp/mould
Medication history

9. Specialist tests Blood tests CT scan Lung function tests
Rheumatoid arthritis and other underlying causes
CT scan
Lung function tests

10. CT pattern

11. Spirometry

12. Making the diagnosis
Interstitial Lung Disease (ILD) Multidisciplinary team (MDT)
Respiratory consultant
Radiology consultant
Histopathology consultant
Specialist nurse
Review history, CT, blood results to decide if a diagnosis can be reached
Sometimes the MDT will recommend further tests

13. Further tests Bronchoscopy? Rheumatology specialist opinion?
Lung biopsy?
THEN back to ILD MDT to agree diagnosis

14. Explaining the diagnosis
Explained by consultant and specialist nurse
Verbal and written information
Action for Pulmonary Fibrosis
British Lung Foundation
Management plan
Drugs
Oxygen assessment
Exercise/rehabilitation
Symptom control/palliation

15. Monitoring Symptoms Pulse oximetry Weight Lung function Repeat imaging
Spirometry
Forced Vital Capacity (FVC)
Repeat imaging
Chest x-ray
CT scans

16. Oxygen assessment 2 ways that oxygen may be prescribed:
Ambulatory (portable)
For patients with limited exercise tolerance shown to desaturate on a walk test (using pulse oximetry)
Only if the patient feels better and walks further with the oxygen
‘Long term’ oxygen therapy (LTOT)
For patients with low oxygen levels even at rest (tested with a blood test)
Prescribed for at least 15 hours per day
Thought to protect the heart

17. Complications of pulmonary fibrosis
Infection
Annual flu jab
Pneumovax
Heart failure
Swollen ankles and worsening breathlessness
Diuretics (‘water tablets’)
Malignancy

18. Summary
Diagnosis of pulmonary fibrosis (and especially what type of pulmonary fibrosis) can be complex
Specialist teams (MDT) should be involved in confirming diagnosis and initial management plan
Follow-up will focus on monitoring for disease progression or complications

19. Questions