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How is pulmonary fibrosis diagnosed and monitored?

How is pulmonary fibrosis diagnosed and monitored?. Dr Paul Beirne. To discuss. Who gets pulmonary fibrosis? Symptoms and signs Radiology Lung function Monitoring over time Oxygen assessment Complications of pulmonary fibrosis. Who gets pulmonary fibrosis?.

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How is pulmonary fibrosis diagnosed and monitored?

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  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 • 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 • 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

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