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? 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

Symptoms Cough Breathlessness on exertion None

Signs Crackles Digital clubbing

Digital clubbing

Initial tests (GP) Chest x-ray Spirometry

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

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

CT pattern

Spirometry

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

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

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

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

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

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

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

Questions