Restrictive lung disease Flashcards
Restriction, physiological definitaion re fvc?
Restrictive lung disease has an FVC of less than 80% of what is expected.
Tidal vol + Inspriatory resere and Expiratory reserve graph. What changes are seen in
Obstructive and Restrictive breathing compared to normal?
Obstructive = increased reserve volume and decreased expiratory reserve volume - whole graph shifts upwards.
Restrictive = reduced reserve volume, reduced vital capacity. Graph visably shrunken and lower doen.
FEV1 marker of, FVC marker of
FEV1 reduced is a marker of obstructive lung disease
FVC reduced is a marker of restrictive (but also may be reduced in obstructive)
Causes of Restrictive lung disease?
Lung Causes: Empysema, Sarcoidosis, Idiopathic Pulmonary Fibrosis, Hypersensitivity Pneumonitis
Pleural causes: Pleural effusion, Pneumothorax, Pleural thickening eg in Mesothelioma.
Skeletal causes: Kyphoscoliosis (curvy spine), Ankylosing Spondylitis, rib fractures, Thoracoplasty,
Muscle/Neuro Amyotrophic lateral sclerosis
Sub Diaphagmatic causes = Obestity, pregnancy,
Where does honeycombing begin and work its way?
Perhipheral to central
What colour is pleural fluid?
Straw coloured
What is Thoracoplasty?
A surgery in the olden days to help treat TB (aerobic), by removing some ribs and deliberatly collapse chest
Where is interstitium?
Between the basement membranes of epitherlium of the alveolar and the endothelium of the capillaries
How many ILD are there? What are they and what can they result in?
More than 200(!) - largely due to the many types of HP.
Can result in fibrosis of the lung (Pulmonary fibrosis)
What is sarcoidosis and histological hallmark?
Systemic granulomatous
Hallmark = Non-caseating granulomas
What is erythema nodosum? What disease?
Sarcoidosis - red raised painful scaly patches on skin - inflammatory cells. Particularly on legs.
Erythema = red Nodosum = bumpy
How do you see if sarcoidosis? ie what investigation primarily?
CXR
Why should sarcoid get optemetry?
Beacuse it can lead to anterior uveitis (inflammation at front of eye).
Can also get inflammation at the back and sides which can lead to loss of vision.
Where do granulomas tend to locate on skin?
Around areas of injury, eg tattoos/scars
Lupus Pernio - what is ?
Disfiguring nasal lesion (Think bluedolf - rudolf the blue nose reindeer) - rare manifestation of chronic sarcoisosis
Who usually gets sarcoidosis?
Generally younger population (U40), women more often than men and worldwise
How to investigate sarcoidosis?
History and exam,
CXR
What is paratrachyl englargement and how des it present on x rays?
Enlargement of the lymph nodes on the sides of the trachea - present as shadowing next to the trachea, above mediastinum in lung.
Stage 1 and 2 disease sarcoidosis (of lung)
So stage 1 is within lymph nodes, stage 2 is spotty lungs on XRays, so lung involvement aswell as the lymph glands
Stage 3 Sarcoisosis of lungs
Larger interstitial change
STAGE 4 sarcoisosis of lungs
Fibrotic change within the lungs
How do you investigate the gland enlargement - what could be a differential diagnosis of lymph enlargement?
many differentials -including TB
Pulmonary function tests
Bloods/urubakysis, ECG/TB skin test/eye exam
If in doubt, what further investigations?
Biopsies - bronchoscopy inc transbronchial biopsies
Remission rates Stage 1-4?
Stage 1 - 55-90%
Stage 2 - 40-70%
Stage 3 - 10-20%
Stage 4- 0% - rriversiable damage has been done!
Treatment ?
Not really any sypmtoms/ no vital organ involvement - stage 1 = nothing
Erythema nodosum (red node patches)/arthalgia (joint stiffness) = NSAIDS
Skin lesions/anterior uvelitis/cough - Topical steroids
Cardiac/neuro/hypercalcaemia/eye not responding to topical = systemic
What lung complications can sarcoidosis lead to?
Bronchiectasis Aspergilloma Haemoptysys Pneumothorax Progressive resp. failure
What is a fibroblastic focus and in what disease is it commonly seen?
swirl of fibroblasts spouting out collagen, seen in Idiopathic pulmonary fibrosis
Who usually presents with IPF and what with? differential diagnosis?
Older men presenting with chronic cough and breathlessness. (can also be women - typically 60-70yo)
CRACKLES in LUNG and CLUBBING
Differential diagnosis
Infection - often thought, no improvement (inc to crackles) on antibiotics
Left Ventricular Failure
What is traction bronchiectasis?
When the fibrosis pulls at the bronchioles/bronchi and makes them larger than they should be
What are the treatment options for IPF?
Not really much :(
Median survival is 3 years :(
BUT some drugs are coming onto the market now which can help SLOW (not stop) the progression of fibrosis eg OAF (oral anti-fibrotic) - Pirfenidone, Nintedanib
HP - nosey questions - why?
To try and figure out if they have been exposed to any organic compounds that could be causing the hypersensitivity. (Including birds, mouldy hay, malt whiskey factories and down duvets)
