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Interstital Lung Disease

Interstital Lung Disease. Nicholas Ashley SHO. Definition – Interstital Fibrosis. Chronic inflammatory condition of the lung parenchyma that has multiple aetiologies but ultimately results in fibrosis of the alveoli and interstitium through fibroblast activation

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Interstital Lung Disease

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  1. Interstital Lung Disease Nicholas Ashley SHO

  2. Definition – Interstital Fibrosis • Chronic inflammatory condition of the lung parenchyma that has multiple aetiologies but ultimately results in fibrosis of the alveoli and interstitium through fibroblast activation • Doesn’t affect the airways, affects the part involved in gaseous exchange Aims • Learn causes of pulmonary fibrosis • Main investigations in pulmonary fibrosis • Treatment strategies

  3. Causes Drugs CTD Sarcoidosis Inorganic (Pneumoconiosis) Idiopathic Organic (EAA)

  4. Clinical Features SOB (Progressive) • HISTORY Onset? Timeframe? Progressive? Order of Sx? Cough (No Sputum) Ex Tolerance Weight Loss After HPCx: Fatigue PMHx Smoking Pets Exposure in Job/Hobby Ask specific meds! If confident ask CTD Sx

  5. Differentials to Consider CCF • Pink frothy sputum • Pillows at night • PMHx HTN/Valve/IHD • PND and Orthopnoea • Ankle swelling • Palpitations BRONCHIECTASIS • Thin copious sputum • ?Severe lung insult in PMHx LUNG CANCER • Weight loss • Smoking/exposure • Routine questioning COPD

  6. EXAMINATION Clubbing BibasalCrackles CorPulmonale Respiratory Effort  Reduced Expansion Cyanosis

  7. Investigations

  8. Treating Idiopathic Pulmonary Fibrosis MDT Difference of Opinion • Corticosteroids • Azathioprine • N-Acetyl Cysteine Smoking Cessation Pulm Rehab Palliate Important LTOT Surgery If the cause isnt idiopathic then treatment varies – as a rule of thumb it often involves starting steroids and/or removing causative agent!

  9. Prognosis • IDIOPATHIC - Generally poor as there is not much that can be done to slow progression • OTHERS - Very variable. Can often slow disease progression but cannot reverse fibrotic changes already present

  10. Case Vignette • 70 year old gentleman presents to you with SOB gradually worsening over the last 8 months • His exercise tolerance has decreased from being able to walk 3 miles to now managing 200 yards before stopping due to SOB • He has no PMHx of note and has smoked 20/day for 40 years • He appears SOB on examination with clubbed fingers and a saturation of 91% OA. Examination reveals fine fixed crackles and no wheeze

  11. Vignette Questions • What is your differential list? • Any specific symptoms or signs to rule in fibrotic lung disease / rule out differentials? • How would you fully investigate this patient – justify your use of each Ix • What would the management plan entail and what would this gentleman’s prognosis be?

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