Clinical Features and Management of Restrictive Lung Disease Flashcards
What is the physiological definition of restriction?
Forced vital capacity less than 80% of the predicted normal
What is a marker of restriction?
Vital capacity
What are causes of restriction?
Lungs
Pleura
Nerve
Muscle
Bone
What are lung causes of restriction?
Interstitial lung diseases
What are examples of interstitial lung diseases?
Idiopathic pulmonary fibrosis
Sarcoidosis
Hypersensitivity pneumonitis
What are pleural causes of restriction?
Pleural effusions
Pneomothorax
Pleural thickening
What are skeletal causes of restriction?
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures
What are muscle causes of restriction?
Amyotrophic lateral sclerosis
What are sub-diaphragmatic causes of restriction?
Obesity
Pregnancy
What should you remember about the causes of restriction?
Not always lung disease
Where is the lung interstitium?
Between the epithelium of the alveoli and the endothelium of the capillary
What does ILD stand up for?
Interstitium lung disease
How many diseases cause the thickening of the interstitium and can result in pulmonary fibrosis?
More than 200
What are the top 3 lung diseases that cause interstitium lung disease?
Sarcoidosis
Idiopathic pulmonary fibrosis
Hypersensitive pneumonitis
What is sarcoidosis?
Multisystem granulomatous disease of unknown cause
What age and sex commonly gets sarcoidosis?
Younger than 40
Women more than men
How do you investigate sarcoidosis?
History and examination
Chest X-ray
Pulmonary function tests
Bloods
What further assessments can be done to investigate sarcoidosis?
Bronchoscopy including transbronchial biopsies and endobronchial ultrasound
What surgical biopsies can be done for sarcoidosis?
Mediastinoscopy
Video assisted thoracoscopic lung biopsy
What does VATS stand up for?
Video assisted thoracoscopic lung biopsy
How should sarcoidosis be treated if mild disease with no vital organ involvement, normal lung function and few symptoms?
No treatment
How should sarcoidosis be treated if erythema nodosum/arthralgia?
NSAIDS
What is erythema nodosum?
Swollen fat under the skin causing red bumps
What is swollen fat under the skin causing red bumps called?
Erythema nodosum
What is arthralgia?
Pain in a joint
What is pain in a joint called?
Arthralgia
How should sarcoidosis be treated if skin lesions and a cough are present?
Topical steroids
How should sarcoidosis be treated if cardiac, neurological and eye disease not responding to topical steroids, or are hypercalcaemia?
Systemic steroids
What are some permanent pulmonary complications that can occur due to sarcoidosis?
Progressive respiratory failure
Bronchiectasis
Aspergilloma
Pneumothorax
What percentage of caucasians die from sarcoidosis?
<1%
What is the typical presentation of idiopathic pulmonary fibrosis?
Chronic breathlessness and cough
Typically 60-70 years old
Clubbed and crackles
What is idiopathic pulmonary fibrosis?
Scarring of the lung
What is scarring of the lung for unknown causes called?
Idiopathic pulmonary fibrosis
What is the median survival time for idiopathic pulmonary fibrosis?
3 years
What are the options for pulmonary fibrosis?
Refer to interstitial lung disease clinic
Palliative care
Transplant
What is hypersensitive pneumonitis?
Lungs become inflammed due to an allergic reaction
What is it called when the lungs become inflammed due to an allergic reaction?
Hypersensitive pneumonitis
