Fibrotic lung disease

• What is fibrotic lung disease also known as?

  Pulmonary fibrosis
• What is pulmonary fibrosis?

  Stiffening of the lung -> decline in lung function = breathing difficulties
• What are some risk factors for developing pulmonary fibrosis?

  Smoking

  Environmental exposures

  Genetics

  Connective tissue diseases
• What is the most common type of fibrotic lung disease?

  Idiopathic pulmonary fibrosis
• What are some signs and symptoms of pulmonary fibrosis?

  Chronic dyspnoea

  Cough

  Clubbing
• When auscultating a patient with pulmonary fibrosis what may you hear?

  Bibasal crackles
• How is a diagnosis of pulmonary fibrosis made?

  HRCT (if inconclusive, lung biopsy)
• What may HRCT show for pulmonary fibrosis?

  Usual interstitial pneumonia
• How is pulmonary fibrosis managed?

  Medication
• What is the main goal for managing pulmonary fibrosis?

  Symptom management

  Slowing disease progression
• What medications/therapies may be used for managing pulmonary fibrosis?

  Antifibrotic drugs - Pirfenidone and nintedanib

  Oxygen therapy

  Pulmonary rehab

  Lung transplant (severe)
• What are some causes of upper fibrosis? (CHARTS)

  Coal workers

  Hypersensitivity pneumonitis

  Ankylosing spondylitis

  Radiation

  TB

  Silicosis
• What are some causes of lower fibrosis?

  Idiopathic

  RA

  SLE

  Systemic sclerosis

  Asbestosis

  Medications - Amiodorone, Methotrexate, Nitrofuratoin
• What is shown on an HRCT for pulmonary fibrosis?

  Ground glass -> honeycombing
• What pattern would spirometry show for pulmonary fibrosis?

  Restrictive pattern