Clinical Features and Management of Restrictive Lung diseases Flashcards
What is the FVC for restrictive disease?
<80% of normal
What are the types of restrictive disease of the lung?
Interstitial lung disease
- Idiopathic pulmonary fibrosis
- Sarcoidosis
- Hypersensitivity pneumonitis
- Honeycombing fibrosis
What are some pleural restrictive lung diseases?
Pleural effusions
Pneumothorax
Pleural thickening
What are some nerve/muscle restrictive diseases?
Amyotrophic lateral sclerosis
Motor neurone disease
What are some bone restrictive diseases?
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures
What are some other causes of restrictive lung disease?
Sub diaphragmatic causes
- Obesity
- Pregancy
What are some other causes of restrictive lung disease?
Sub diaphragmatic causes
- Obesity
- Pregnancy
What is the histological hallmark of sarcoidosis?
non-caseating granuloma
What is the rash caused by sarcoidosis called and what does it look like?
Erythema nodosum
- Red
- Bumpy
- Painful
- Self limiting
What are some other symptoms of sarcoidosis?
Inflammation of the eye
Skin lesions
Scalp lesions
Granulomas located around scars
Lupus pernio
Who is more at risk of sarcoidosis?
Women
Young people
How is sarcoidosis investigated? (11)
History
Examination
CXR
CT
Lymphadenopathy
Pulmonary function tests
Bloods
Urinalysis
ECG
TB skin test
Eye exam
What’s a further assessment you can do for sarcoidosis?
Tissue diagnosis
- Bronchoscopy
- Surgical biopsies
What is the remission rate for each stage of sarcoidosis?
Stage 1 = 55-90%
Stage 2 = 40-70%
Stage 3 = 10-20%
Stage 4 = 0%
What does mild sarcoidosis look like?
No vital organ involvement
Normal lung function
Few symptoms
How do you treat mild sarcoidosis?
No treatment - watchful waiting
How do you treat erythema nodosum/arthralgia?
NSAIDs
(non steroid anti-inflammatory drugs)
How do you treat skin lesions/anterior uveitis/cough?
Topical steroids
What is treated with systemic steroids? (5)
Cardiac
Neurological
Eye disease not responding to topical treatment
Hypercalcaemia
What are some complications of sarcoidosis? (5)
Progressive respiratory failure
Bronchiectasis
Aspergilloma
Haemoptysis
Pneumothorax
How does IPF present?
Chronic breathlessness
Cough
Typically 60-70 years old
Failed Rx for LVF or infection
Clubbed fingers
Crackles that dont go away
What would you see on a chest X-ray for IPF?
Shadowing at base of lung due to fibrosis
What are the options for someone diagnosed with IPF?
Refer to interstitial lung disease (ILD) clinic
Medical options
Surgical options
What are some medical options for IPF?
Oral anti-fibrotic (OAF)
- Pirfenidone
- Nintedanib
Palliative care
