Clinical features and restrictive lung disease Flashcards
what is the physiological definition of restriction?
FVC <80% of the predicted normal
what diseases affect the lungs, that are also ILDs? (diseases that cause restrictive disorders)
- idiopathic pulmonary fibrosis
- sarcoidosis
- hypersensitivity pneumonitis
what diseases affect the pleura? (diseases that cause restrictive disorders)
- pleural effusions
- pneumothorax
- pleural thickening
what diseases have skeletal causes? (diseases that cause restrictive disorders)
- kyphoscoliosis
- ankylosing spondylitis
- thoracoplasty
- RIB fractures
what diseases have muscles causes? (diseases that cause restrictive disorders)
- amyotrophic lateral sclerosis
what sub-diaphragmatic have skeletal causes? (diseases that cause restrictive disorders)
- obesity
- pregnancy
what are ILDs? (HINT: there’s a shit tonne of them)
> 200 diseases causing thickening of the interstitium and can result in pulmonary fibrosis (disease linked to environment)
what is sarcoidosis?
multisystem granulomatous disease of unknown cause
who is most affected by ILDs? (not IPF)
adults <40, F>M, worldwide
how do you investigate ILDs?
history, exam, CXR + pulmonary function tests (bloods/ urinalysis, ECG, TB skin test, eye exam) + further assessments (bronchoscopy including transbronchial biopsies and endobronchial ultrasound
what do you need for a surgical biopsy? (to investigate ILD)
- mediastincoscopy
- video assisted thoracoscopic lung biopsy (VATS)
how should ILDs be treated if mild disease, no vital organ involvement, normal lung function, few symptoms? (not IPF)
no treatment
how should ILDs be treated if erythema nodosum/ arthralgia? (not IPF)
NSAIDs
how should ILDs be treated if skin lesions/ anterior uveitis/ cough? (not IPF)
topical steroids
how should ILDs be treated if cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia? (not IPF)
systemic steroids