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)
What are complications of hypersensitivity pneumonitis (extrinsic allergic alveolitis)? (2)
- cor pulmonale
- type 1 respiratory failure
What name is given to a lung affected by interstitial lung disease?
honeycomb lung
What are pleural causes for restrictive lung disease? (3)
- pleural effusion
- pneumothorax
- pleural thickening
What can cause pleural thickening? (2)
- asbestos exposure
- pneumonia
What is a pleural effusion?
- accumulated liquid in pleural space
- can be divided by their protein concentration into transudates (<25g/l) and exudates (>35g/l)
What is blood accumulated in pleura called?
haemothorax
What is pus accumulated in pleura called?
empyema
What is chyle (lymph with fat) accumulated in pleura called?
chylothorax
What are transudates? (proteins found in pleural effusion)
- due to increased venous pressure (increased hydrostatic pressure); which can be as a result of cardiac failure, constrictive pericarditis or fluid overload
- due to hypoproteinaemia which can be due to cirrhosis, nephrotic syndrome or malabsorption
In general, transudates are as a result of an imbalance between production and removal of pleural fluid in lungs
Disease of which organs can result in an imbalance filling the pleural spaces with transudates? (3)
- heart (venous obstruction)
- liver (decrease osmotic pressure)
- kidneys
e. g. heart failure increase pressure in small blood vessels responsible for removing pleural fluid causing transudate to leak into pleural space
What are exudates? (proteins found in pleural effusion)
- mostly due to increased leakiness of pleural capillaries secondary to infection, inflammation or malignancy
- result of disease or damage to pleura itself
- fluid is secreted and forced out from the pleura itself and the pleural space fills with cloudy fluid containing proteins and cell debris
- INFLAMMATION response
Are exudates or transudates higher in protein content?
exudates
What are the skeletal causes for restrictive lung disease?
- kyphoscoliosis
- ankylosing spondylitis
- thoracoplasty
What is the main skeletal cause for a restrictive lung?
rib fracture
What is the main muscle cause for a restrictive lung?
amyotrophic lateral sclerosis (ALS); muscle pump driving the lungs isn’t working
What are 2 sub diaphragmatic causes for a restrictive lung?
- obesity
- pregnancy
How many diseases are there that cause thickening of the interstitium?
over 200 diseases (and cause pulmonary fibrosis)
What is the histological hallmark for sarcoidosis? (interstitial lung disease)
non caseating granuloma (cheese-like appearance and common in TB granulomas)
How is sarcoidosis investigated? (8)
- history and exam
- chest x ray
- pulmonary function tests
- blood
- urinalysis
- ECG
- TB skin test
- eye exam
What further assessment is sometimes needed to diagnose sarcoidosis? (2)
- Bronchoscopy including transbronchial biopsies
- Endobronchial ultrasound
(cells can be examined)
What are 2 types of surgical biopsies sometimes needed to diagnose a restrictive lung disease if there is a struggle for diagnosis?
- mediastinoscopy
2. video assisted thoracoscopic lung biopsy (VATS)
What is the remission rate of Stage 1 sarcoidosis? ( does a chest x ray affect prognosis?)
55-90% remission rate
most patients present at this stage
What is the remission rate of stage 2 sarcoidosis? (does a chest x ray affect prognosis?)
40-70% remission rate
What is the remission rate of stage 3 sarcoidosis? (does a chest x ray affect prognosis?)
10-20% remission rate
What is the remission rate of stage 4 sarcoidosis? (does a chest x ray affect prognosis?)
0% remission rate
How should sarcoidosis be treated with: mild disease, no vital organ involvement, normal lung function and a few symptoms?
No treatment
How should sarcoidosis be treated with: erythema nodosum (red lumps or nodules under skin esp. shins) and arthralgia?
NSAIDs ; non-steroidal anti-inflammatory drugs
e.g. aspirin, ibuprofen or naproxen
How should sarcoidosis be treated with: skin lesions, anterior uveitis and cough?
Topical steroids
How should sarcoidosis be treated with: cardiac, neurological problems, eye disease not responding to topical treatment, hypercalcaemia?
Systemic steroids
What is the prognosis for sarcoidosis?
“probably ok”
very few Caucasian’s die (<1%)
What percentage of people will sustain permanent pulmonary or extra pulmonary complications?
10-20%
What main respiratory complications can arise from sarcoidosis? (5)
- progressive respiratory failure
- bronchiectasis
- aspergilloma (fungus ball)
- haemoptysis
- pneumothorax
What is a typical presentation for idiopathic pulmonary fibrosis?
- chronic breathlessness and cough
- typically 60-70 years old (more common in men)
- failed treatment for LVF (left ventricular failure) or infection
- clubbed and crackles
What is median survival rate for idiopathic pulmonary fibrosis?
3 years
What are treatment options for idiopathic pulmonary fibrosis? (4)
- oral anti-fibrotic; Pirfenidone and Nintedanib
- pallative care
- surgery; transplant
- refer to ILD clinic
