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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 • 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 Causes Drugs CTD Sarcoidosis Idiopathic Inorganic (Pneumoconiosis) Organic (EAA) Idiopathic Drugs Organic “Idiopathic Interstitial Pneumonitis” Idiopathic Pulmonary Fibrosis Methotrexate Pigeon Fancier Desquamative Interstitial Pneumonia Nitrofurantoin Farmers Acute Interstitial pneumonitis Amiodarone Hot Tub or Malt Workers Inorganic CTD Sarcoidosis Coal Minors Rheumatoid Arthritis OWN CATEGORY Asbestosis Systemic Sclerosis Silicosis Vasculitidies Clinical Features • HISTORY Onset? Timeframe? Progressive? Order of Sx? After HPCx: PMHx Smoking Pets Exposure in Job/Hobby Ask specific meds! If confident ask CTD Sx Differentials to Consider CCF • • • • • • BRONCHIECTASIS • Thin copious sputum • ?Severe lung insult in PMHx LUNG CANCER • Weight loss • Smoking/exposure COPD • Routine questioning Pink frothy sputum Pillows at night PMHx HTN/Valve/IHD PND and Orthopnoea Ankle swelling Palpitations • EXAMINATION Investigations BLOODS MICRO IMAGING SPECIAL FBC, U&E, LFT, Ca, ACE Sputum MCS CXR Spirometry RF, antiCCP, ANA TB Culture Echo BAL HRCT Biopsy T-Spot ABG Treating Idiopathic Pulmonary Fibrosis MDT Smoking Cessation Difference of Opinion • Corticosteroids • Azathioprine • N-Acetyl Cysteine Pulm Rehab LTOT Important Palliate Surgery If the cause isnt idiopathic then treatment varies – as a rule of thumb it often involves starting steroids and/or removing causative agent! 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 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 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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