26. Clinical Features and Management of Restrictive Lung Disease Flashcards
Define restriction (restrictive lung disease).
- forced vital capacity < 80% of the predicted normal
What occurs to ALL lung volumes in restrictive disease? (total lung capacity, vital capacity, expiratory volume, ERV etc)
They ALL decrease (all become smaller)
What volumes decrease in OBSTRUCTIVE lung disease?
- expiratory reserve volume (ERV)
- vital capacity (VC)
All are to do with expiration
What volumes increase in OBSTRUCTIVE lung disease?
- total lung capacity (TLC)
- functional residual capacity (FRC); ERV + RV
- reserve volume (RV)
Which volume “type” is a marker of restriction?
Vital capacity
What is abnormal vital capacity?
VC<80% is abnormal
What technique is key for diagnosing restrictive lung disease?
spirometry
What are the lung causes for restrictive lung disease?
interstitial lung diseases
Which regions of the body can lead to a restrictive lung disease? (5)
- lungs
- pleura
-nerve or muscle - bone
-other
(from inside to outside)
What are 3 main interstitial lung disease which lead to restrictive lung disease?
- idiopathic pulmonary fibrosis
- sarcoidosis
- hypersensitivity pneumonitis
What occurs during idiopathic pulmonary fibrosis? (type of interstitial idiopathic pneumonia)
- alveoli become damaged and increasingly scarred
- causes lungs to become stiff
- commonest cause of interstitial lung disease
- inflammatory cell infiltrate with pulmonary fibrosis of unknown cause
What is another name for idiopathic pulmonary fibrosis?
cryptogenic fibrosing alveolitis
What are the common symptoms for idiopathic pulmonary fibrosis? (5)
- malaise
- exertional dyspnoea
- dry cough
- weight loss
- arthralgia (joint pain)
What are common signs for idiopathic pulmonary fibrosis? (3)
- cyanosis
- finger clubbing
- fine end-respiratory crepitations (crackles)
What complications can arise due to idiopathic pulmonary fibrosis?
- increased risk for lung cancer
- respiratory failure
What occurs during sarcoidosis?
- A multisystem granulomatous disorder of unknown cause
- Granulomas (red and swollen tissues) develops on organs around body (usually affects lungs and skin)
What populations are more at risk for sarcoidosis? (90% of sarcoidosis is pulmonary and 25% is skin)
- prevalence highest in N.Europe
- more common in women than men
- usually affects adults aged 20-40 years old
- Afro-Caribbeans more likely to be affected than Caucasians
What are common symptoms for sarcoidosis? (5)
- dyspnoea
- persistent dry cough
- arthralgia
- malaise
- abnormal heart rhythm and chest pain (but not always)
Why is sarcoidosis difficult to diagnose straight away?
- often presents asymptomatic
- 20-40 discovered incidentally on chest x rays
What is the treatment for sarcoidosis? (3)
- Most recover spontaneously and don’t need treatment
- Acute sarcoidosis requires bed rest
- Prednisolone steroid tablets sometimes used
What is the treatment for idiopathic pulmonary fibrosis?
No treatment currently but medication can relieve pain and symptoms and slow progression
(e.g. lifestyle changes, pulmonary rehabilitation, O2 mask, medication to reduce scarring e.g. pirfenidone or nintedanib or lung transplant)
What is hypersensitivity pnuemonitis? (also called extrinsic allergic alveolitis)
- individuals are sensitised to inhaled allergens (eg. fungal spores or avian proteins)
- alveoli are infiltered with inflammatory cells (acute phase)
- granuloma and acute bronchiolitis can occur (chronic phase)
What are some of the symptoms of hypersensitivity pneumonitis (extrinsic allergic alveolitis)? (6)
- fever and rigors
- myalgia (muscle pain)
- dry cough
- dyspnea on exertion
- weight loss
- crackles (no wheeze)