csim2.94 – interstitial lung disease

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CSIM2.94 – INTERSTITIAL LUNG DISEASE Interstitial space; space between alveoli, capillaries, and airways. Interstitial’ means the disease affects the ‘interstitium’, a lace-like network of tissue that supports the alveoli (air sacs) in your lungs. An imprecise term for a range of diseases which affect the lung parenchyma with cellular infiltration of the alveoli, interstitium and distal airways, and which may progress to fibrosis When you have ILD, inflammation or scar tissue builds up in your lungs, making them thick and hard. This build-up of scar tissue is called fibrosis. As your lungs become stiffer and lose their elasticity, they are less able to take oxygen from the air you breathe. People with ILD can feel breathless from simple everyday activities like walking. Coughing is another common symptom. Idiopathic Pulmonary Fibrosis Common in clinical practice Often serious Very often found in OSCE exams! Often misdiagnosed as; o Cardiac failure o Asthma/COPD o Pneumonia o Bronchiectasis. Presentation Male,

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CSIM stage 4

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CSIM2.94 Interstitial Lung Disease Interstitial space; space between alveoli, capillaries, and airways. Interstitial means the disease affects the interstitium, a lace-like network of tissue that supports the alveoli (air sacs) in your lungs. An imprecise term for a range of diseases which affect the lung parenchyma with cellular infiltration of the alveoli, interstitium and distal airways, and which may progress to fibrosis When you have ILD, inflammation or scar tissue builds up in your lungs, making them thick and hard. This build-up of scar tissue is called fibrosis. As your lungs become stiffer and lose their elasticity, they are less able to take oxygen from the air you breathe. People with ILD can feel breathless from simple everyday activities like walking. Coughing is another common symptom.

Idiopathic Pulmonary Fibrosis Common in clinical practice Often serious Very often found in OSCE exams! Often misdiagnosed as; Cardiac failure Asthma/COPD Pneumonia Bronchiectasis. Presentation Male, SOB, dry cough, smoker, o/e: RR high, O2 saturation: lowFinger clubbing, peripheral cyanosis, fine crackles bi-basally, reduced expansion, Fine = Fibrosis Dry cough Restrictive PhysiologyCoarse = Bronchectasis Wet productive cough Obstructive Physiology

Investigations 1. History taking: is it really idiopathic? a. Environmental/occupational exposures? i.e. dusts, asbestos, petsb. Drug hx i.e. methotrexatec. Connective tissue dx i.e. RAd. Failure to respond to treatment for other conditions. 2. CXR3. Lung function; Spirometry 4. ABGTreatment Nothing, supportive care i.e. O2/rehab Pirfenidone Recruit to clinical trials Transplant. Sarcoid Multisystem granulomatous disease of unknown cause primarily affecting the lung Bilateral Hilar/mediastinal Lymphadenopathy Sarcoid TB Lymphoma Ca Tx: none, steroid if required Hypersensitivity Pneumonitis/ Extrinsic Allergic Alveolitis Example of antigens Farmer's lung Saw mill worker's lung Bird fancier's lung Mushroom workers lung Malt workers lung Humidifier lung Cheese washer's lung Suberosis

Presentations Cough, SOB, systemic sx such as fever, weight loss o/e: Crackles and also wheeze and squeks Usually suffice to have appropriate exposure, positive antibody response and clinical/radiological pictureTreatment Antigens avoidance Steroids