Restrictive Lung Disease Flashcards
What is the physiological definition of restrictive lung disease?
FVC is <80% of normal
What volumes are affected in restrictive lung disease?
All are reduced - VC, RV, ERV, IC, TLC and TV
What is the marker of restrictive lung disease?
Vital capacity
VC < 80% is abnormal
What is used to determine FVC?
Spirometry
What are some interstitial lung diseases?
Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis
What are some pleural causes of restrictive lung disease?
Pleural effusions
Pneumothorax
Pleural thickening
Explain pleural effusions
There is an excessive collection of fluid in the pleural cavity
Has restricted pattern of lung airflow
Explain a pneumothorax
A collapsed lung that occurs when air enters into the pleural cavity
This causes chest pain and breathlessness
Explain pleural thickening
Increase of bulkiness of one or both of the pulmonary pleura
Can be due to asbestos exposure
TB can also cause pericardium thickening
What are some skeletal causes for restrictive lung disease?
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures
What is kyphoscoliosis?
An abnormality of the spine causing excessive curvature of the upper back. This causes pain and stiffness
What is ankylosing spondylitis?
A type of arthritis in the spine, causing inflammation and gradual fusing of the vertebrae
What is a thoracoplasty?
Thoracoplasty is a surgical technique initially designed to permanently collapse tuberculous cavities by resection of ribs from the chest wall
Volume in right is one side is increased and calcification can occur
Explain rib fractures and restrictive lung condition?
If sore then cant expand rib
If in CF then can be dangerous as can bring up a lot of sputum
Describe amyotrophic lateral sclerosis and restrictive lung disease?
This is caused by muscles
Muscles cant inflate lungs
What are some sub-diaphragmic causes of restrictive lung disease?
Obesity and pregnancy
Where is the interstitium?
It is between the epithelium of the alveolus and endothelium of the capillary
Describe the interstitium?
Is very thin and if thickened can cause shortness of breath
What are interstitial lung diseases?
There are over 200 diseases causing thickening of the interstitium and can result in pulmonary fibrosis
What is sarcoidosis?
Multisystem granulomatous disease of unknown cause
Granuloma is a cluster of inflammatory cells
What is the histological hallmark of sarcoidosis?
Non-caseating granuloma
What is Erythema nodosum?
Is reddish, painful, tender lumps most commonly located in the front of the legs
Is skin inflammation
Patients with sarcoidosis can present with this rash
What is anterior uveitis?
Inflammation of the iris and ciliary body - not middle of eye
Painful red eye
Describe skin lesions and sarcoidosis?
Skin lesions are granulomatous so show hallmark of sarcoidosis
Where do granulomas tend to be located?
At areas of skin trauma - scars or tattoos
What is lupus pernio?
Is a nasal lesion which can be a clinical presentation of chronic sarcoidosis
Treated with steroid injections and other therapies
Who usually gets sarcoidosis?
Adults under 40 years old
More women then men
Happens world wide
How do we investigate sarcoidosis?
History and exam
CXR
Then pulmonary function tests, bloods, urinalysis, ECG, TB skin test and eye exam
What would further investigation of sarcoidosis involve?
Bronchoscopy including transbronchial biopsies and endobronchial US
What surgical biopsies may be needed in sarcoidosis?
Mediastinoscopy
Video assisted thoracoscopy lung biopsy (VATS) - uncommon
Describe stage 1 -4 of sarcoidosis on CXR?
1 - bulky lymph glands - paratracheal nodes
2 - spotty lungs
3 - interstitial change
4 - fibrotic advancement
Does stage affect prognosis?
Stage 1 has highest of 55-90%
Then lower is stage 4 is 0% as now fibrotic change which is irreversible
How should sarcoidosis be treated in patient with mild disease, no vital organ involvement and normal lung function?
No treatment
How should you treat a patient with erythema nodosum/ arthralgia?
NSAIDs - non-steroidal anti-inflammatory drugs
How would you treat a patient with skin lesions/ anterior uveitis or cough?
Tropical steroids rather than systemic drugs
What is the treatment for a patient who has cardiac, neurological sarcoid, eye disease not responding to topical Rx?
Systemic steroids ex. prednisolone
Also used if patient has hypercalcaemia
What are some respiratory complications of sarcoidosis?
Progressive resp. failure
Bronchiectasis
Aspergilloma can give haemoptysis or pneumothorax
What is idiopathic pulmonary fibrosis?
Is chronic scarring lung disease and breathing becomes increasingly more difficult
Gives the honeycomb lung
Has fibroblastic focus - collagen factory
What is the typical presentations of IPF?
Chronic breathlessness, cough, clubbed and crackles
Failed prescription for LVF or infection
Typically 60-70 can commoner in men
What are the options of management for a patient with IPF?
Refer to ILD clinic
Medical options - oral anti-fibrotic (prifenidone, nintedanib) and palliative care
Transplant
What is hypersensitivity pneumonitis?
When lungs develop an immune response to something you breath in and causes inflammation of lung tissue
What are the causes of hypersensitivity pneumonitis?
Birds and pigeons
Hay
Malted whiskey factory - lungs reacting against factory
