Clinical Features and Management of Restrictive Lung Disease Flashcards
Define RESTRICTION
Forced vital capacity <80% of normal
Causes of restriction
- Lungs
- Pleura
- Nerve or muscle
- Bone
- Other
LUNG causes of restrictive lung disease
‘Interstitial’ lung disease
- Idiopathic pulmonary fibrosis
- Sarcoidosis
- Hypersensitivity pneumonitis
PLEURAL causes of restrictive lung disease
- Pleural effusion
- Pneumothorax
- Pleural thickening
SKELETAL causes of restrictive lung disease
- Kyphoscoliosis
- Ankylosing spondylitis
- Thoracoplasty
- Rib fracture
MUSCLE causes of restrictive lung disease
Amyotrophic lateral sclerosis
SUB-DIAPHRAGMATIC causes of restrictive lung disease
- Obesity
- Pregnancy
What are LDLs
> 200 diseases causing thickening of the interstitium and can result in pulmonary fibrosis
What is sarcoidosis?
Multisystem granulomatous disease of unknown cause
Histological hallmark - non-caseating granuloma
Who gets sarcoidosis?
- Adults < 40
- Women > men
- World-wide
How do you investigate sarcoidosis?
- History and exam
- CXR
- Pulmonary function tests
- Bloods/urinalysis/ECG/TB skin test/eye exam
- BRONCHOSCOPY
~ Including transbronchial biopsies and endobronchial ultrasound
How should sarcoidosis be treated?
- Mild disease, no vital organ involvement, normal lung function, few symptoms = NO TREATMENT
- Erythema nodosum / arthralgia = NSAIDs
- Skin lesions / anterior uveitis / cough = TOPICAL STEROIDS
- Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia = SYSTEMIC STEROIDS
Typical presentation of idiopathic pulmonary fibrosis
- Chronic breathlessness and cough
- Typically 60-70 years old, commoner in men
- Failed Rx for “LVF” or infection
- Clubbed & crackles
Options for idiopathic pulmonary fibrosis
- Median survival is 3 years
- Refer to ILD clinic (Wed, Clinic C)
- Medical options – OAF (oral anti-fibrotic) – Pirfenidone, Nintedanib, palliative care
- Surgical option – transplant
