Clinical Features and Management of Restrictive Lung Diseases Flashcards
What is the physiological definition of restriction?
Forced vital capacity <80% of normal
What is a marker of restriction?
Vital capacity
What is used to determine restriction rates?
Spirometry
What vital capacity value is considered to be abnormal?
<80%
Where might there be restriction?
Lungs
Pleura
Muscle/nerve
Bone
Other
(think staring in the middle and work your way out).
List some interstitial lung diseases.
Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis
List some of the pleural causes of restriction
Pleural effusion
Pleural thickening
Pneumothorax
List some of the skeletal causes of restriction.
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures
What is Thoracoplasty?
Surgery removing some rib segments.
->don’t necessarily need to know, just to aid understanding
What is Ankylosing spondylitis ?
Inflammation of the spine.
->don’t necessarily need to know, just to aid understanding
What is Kyphoscoliosis?
Abnormal curvature of the spine.
->don’t necessarily need to know, just to aid understanding
Name a muscle causes of restriction.
Amyotrophic lateral sclerosis (ms)
Name some sub-diaphragmatic causes of restriction.
Obesity
Pregnancy
What is the interstitium?
Tiny space between the epithelium of the alveolus and the endothelium of the capillary
What can happen to a patient if there is an abnormality in the interstitium?
Can cause breathlessness
What happens to the Interstitium to cause breathlessness in a patient?
It becomes thickened/enlarged.
What are interstitial lung diseases?
Diseases causing thickening of the interstitium and can result in pulmonary fibrosis
What is sarcoidosis?
Interstitial lung disease
Multisystem granulomatous disease of unknown cause
What is the histological landmark in sarcoidosis?
Non-caseating granulomas (without necrosis)
What is a classical presentation for someone with sarcoidosis?
Red, raised, painful legion on the legs
Where will granulomas often locate?
Areas of skin trauma e.g. scars, tattoos
Who tends to get sarcoidosis?
Adults <40
Women get it more than men
What are the investigations for sarcoidosis?
History and examination
Chest x-ray
Which surgical biopsies may be required for somebody with sarcoidosis?
Mediastinoscopy
Video assisted thoracoscopic lung biopsy (VATS)
What is the treatment for someone with sarcoidosis when they present as following-
Mild disease, no vital organ involvement, normal lung function, few
symptoms
No treatment
What is the treatment for someone with sarcoidosis when they present as following-
Erythema nodosum / arthralgia
NSAIDs
What is the treatment for someone with sarcoidosis when they present as following-
Skin lesions / anterior uveitis / cough
Topical steroids
What is the treatment for someone with sarcoidosis when they present as following-
Cardiac, neurological, eye disease not responding to topical Rx,
hypercalcaemia
Systemic steroids
What are some of the respiratory complications which may arise in someone with sarcoidosis?
Progressive respiratory failure
Bronchiectasis
Aspergilloma, haemoptysis, pneumothorax
What is the typical presentation for someone with Idiopathic pulmonary fibrosis (IPF)?
Chronic breathlessness & cough
Clubbed & crackles
Describe the people which are more likely to develop IPF.
60-70 years old
More common in men
What is the average survival rate of IPF?
3 years
Name some treatment options for those w IPF which although cannot cure, can slow the disease.
Drugs
Palliative care
Surgical transplant
What is hypersensitive pneumonitis?
Developing an immune response to something breathed in and causing inflammation of the airways.
What can hypersensitive pneumonitis be caused by?
Birds- by breathing in particles from feathers of birds/bird droppings
Gran/stray/hay- breathing in a mould which may grow on it
