Clinical Features and Management of Restrictive Lung diseases Flashcards
What is the physiological definition of restriction ?
FVC <80% of normal
What is the marker of restriction ?
Vital capacity- marker of restriction
Spirometry is key
What are the lung causes of Interstitial lung diseases ?
Interstitial lung disease:
- idiopathic pulmonary fibrosis - sarcoidosis - hypersensitivity pneumonitis
What are the pleural causes of restrictive lung disease ?
Pleural effusions
Pneumothorax
Pleural thickening
What are the skeletal causes of restrictive lung diseases ?
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures - soreness
What are the muscular and nerve causes of restrictive lung disease ?
Amyotrophic lateral sclerosis
What are the sub-diaphragmatic causes of restrictive lung disease ?
Obesity
Pregnancy
What is sarcoidosis ?
Multisystem granulomatous disease of unknown cause
What is the histological hallmark of sarcoidosis ?
Non-caseating granuloma
What are the clinical signs of sarcoidosis ?
Erythema nodosum - granulomas often locate to scar tissue
Who gets sarcoidosis ?
Adults <40yr
F>M
Worldwide
How do you investigate sarcoidosis ?
History and exam
CXR
Bronchoscopy including transbronchial biopsies and endobronchial ultrasound
Pulmonary function tests
Bloods / urinalysis / ECG / TB skin test / eye exam
Surgical biopsies:
- mediastinoscopy
- Video assisted thoracoscopic lung biopsy
What is the remission rates for stage 1-4 sarcoidosis ?
Stage 1 - 55-90%
Stage 2 - 40-70%
Stage 3 - 10-20%
Stage 4 - 0%
When would you provide no treatment for Sarcoidosis ?
Mild disease, no vital organ involvement, normal lung function, few symptoms
When would you treat sarcoidosis with NSAIDs?
Erythema nodosum / arthralgia
When would you treat sarcoidosis with Topical steroids ?
Skin lesions / anterior uveitis / cough
When would you treat sarcoidosis with Systemic Steroids ?
Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia
What is prognosis ?
10-20% sustain permenant pulmonary or extrapulmonary complications
What is the typical presentation for idiopathic pulmonary fibrosis ?
Chronic breathlessness & cough Typically 60-70 yrs old M>F Failed Rx for LVF or infection Clubbed & crackles
What are the options for Idiopathic pulmonary fibrosis ?
Median survival - 3 years
Refer to ILD clinic
Medical options- OAF (oral anti-fibrotic)- Pirfenidone
Surgical option- treatment
What initiates hypersensitivity pneumonitis ?
Allergens from things like, pigeons, mould, farms etc
