Clinical Features and Management of Restrictive Lung Disease Flashcards
What is the physiological definition of restriction?
FVC<80% of the predicted normal
What happens to lung volumes and capacities in restrictive lung disease?
All values decrease
What is a marker of restriction?
Vital capacity
What investigation is key in diagnosing restrictive lung disease?
Spirometry
What are the 3 main interstitial lung diseases?
- Idiopathic pulmonary fibrosis
- Sarcoidosis
- Hypersensitivity pneumonitis
What pleural causes of restrictive lung diseases are there?
- Pleural effusion
- Pneumothorax
- Pleural thickening
What skeletal causes of restrictive lung disease are there?
- Kyphoscoliosis
- Ankylosing spondylitis
- Thoracoplasty
- Rib fractures
What muscular causes of restrictive lung disease are there?
- Amyotrophic lateral sclerosis
- Many neuromuscular disorders
What sub-diaphragmatic causes of restrictive lung disease are there?
- Obesity
- Pregnancy
What is the interstitium?
The space between the epithelium of the alveolus and the endothelium of the capillary
What are interstitial lung diseases?
More than 200 diseases that cause the thickening of the interstitium and can result in pulmonary fibrosis
What is sarcoidosis?
Multisystem granulomatous disease of unknown cause
What is the histological hallmark of sarcoidosis?
Non-caseating granuloma
Who does sarcoidosis mainly affect?
- Adults under 40
- Women more than men
- World-wide problem
How is sarcoidosis investigated?
- History
- Examination
- Chest X-ray
- Pulmonary function tests
- Bloods
- Urinalysis
- ECG
- TB skin test
- Eye exam
How can sarcoidosis be further investigated?
- Bronchoscopy
- Transbronchial biopsies
- Endobronchial ultrasound
What surgical biopsies might be carried out for sarcoidosis?
- Mediastinoscopy
- Video assisted thoracoscopic lung biopsy
How does remission rate vary across the stages of sarcoidosis?
- Stage 1: 55-90%
- Stage 2: 40-70%
- Stage 3: 10-20%
- Stage 4: 0%
How should mild sarcoidosis (no vital organ involvement, normal lung function, few symptoms) be treated?
No treatment
How should sarcoidosis with erythema nodosum and arthralgia be treated?
- NSAIDs
- (Some treat with prednisolone)
How should sarcoidosis with skin lesions, anterior uveitis and cough be treated?
Topical steroids
How should sarcoidosis with cardiac, neurological, eye disease not responding to topical Rx, and/ or with hypercalcaemia be treated?
Large doses of systemic steroids
What pulmonary/ extra pulmonary complications do some sustain from sarcoidosis?
- Progressive respiratory failure
- Bronchiectasis
- Aspergilloma
- Haemoptysis
- Pneumothorax
What is the typical presentation for idiopathic pulmonary fibrosis?
-Chronic breathlessness
-Cough
Failed Rx for LVF infection
-Clubbing
-Crackles
Who is usually affected by idiopathic pulmonary fibrosis?
- Commoner in men
- Aged 60-70
What is the median survival for idiopathic pulmonary fibrosis?
3 years
What options are there for management of idiopathic pulmonary fibrosis?
- Referral to ILD clinic
- Oral anti-fibrotic (Pirfenidone, Nintedanib)
- Palliative care
- Lung transplant in those aged under 65
What are some causes of hypersensitivity pneumonitis?
- Birds
- Hay
