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What the GP should know about ‘Fibrosing alveolitis’

What the GP should know about ‘Fibrosing alveolitis’. Dr Paul Beirne. ‘Fibrosing Alveolitis’. What is it? Symptoms Signs History taking Screening tests Securing a diagnosis Prognosis and treatment. IPF = Idiopathic Pulmonary Fibrosis (Cryptogenic Fibrosing Alveolitis (CFA)). Symptoms.

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What the GP should know about ‘Fibrosing alveolitis’

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  1. What the GP should know about ‘Fibrosing alveolitis’ Dr Paul Beirne

  2. ‘Fibrosing Alveolitis’ • What is it? • Symptoms • Signs • History taking • Screening tests • Securing a diagnosis • Prognosis and treatment

  3. IPF = Idiopathic Pulmonary Fibrosis (Cryptogenic Fibrosing Alveolitis (CFA))

  4. Symptoms • Dyspnoea • Cough • Constitutional • None

  5. Signs • Digital clubbing 25-50% • Bibasilar fine late inspiratory crackles (‘Velcro’ crackles) • Look for: • Cyanosis • Cor pulmonale (raised JVP, peripheral oedema) • Rheumatological signs • Evidence of malignancy

  6. History • Drugs • Occupational • Asbestos • Silica or coal dust • Metals • Environmental • Smoking • Birds, moulds/damp (e.g. compost, hay) • Family • Rheumatological • Arthralgia • Raynauds • Dry eyes or mouth

  7. Screening Tests • Spirometry • CXR

  8. Spirometry in Pulmonary Fibrosis • Restrictive • FVC greatly reduced • FEV1 reduced • FEV1/FVC ratio increased to > 80% • Pitfalls • Smokers • Sarcoidosis etc.

  9. CXR • Normal • Peripheral basal reticular shadowing suggests Idiopathic Pulmonary Fibrosis • Other findings in other pulmonary fibrotic diseases include • reticulonodular shadowing • ground glass shadowing • consolidation • Accurate diagnosis on CXR alone is not possible

  10. Securing a diagnosis • History • Examination • HRCT chest • Lung function • Spirometry • Lung volumes • Gas transfer • Bronchoscopy • Bronchoalveolar lavage • Transbronchial lung biopsies • Surgical lung biopsy??? • VATS

  11. Prognosis • Idiopathic Pulmonary Fibrosis • Inexorable slow decline punctuated by periods of accelerated decline • Median time from diagnosis to death 2.5 years • Non-Specific Interstitial Pneumonitis (NSIP) • Variable prognosis with recovery or stability on treatment being usual; minority progress to death

  12. Treatment • Steroids • Steroid sparing agents • Azathioprine • Mycophenolate • Antioxidants • NAC • For Idiopathic Pulmonary Fibrosis, current best treatment: • NONE • ‘Triple therapy’ with low dose prednisolone, azathioprine 2 mg/kg and NAC • Early referral for transplant

  13. Summary • Pulmonary fibrosis thickens the alveolar wall and impairs gas exchange • Presents with dyspnoea and cough • Commonest signs are clubbing and crackles • Spirometry often restrictive • CXR usually abnormal • Accurate diagnosis in secondary care is essential as prognosis and treatment vary markedly between different diseases

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