Clinical Features and Management of Restrictive Lung Diseases

Question 1

Name causes of restriction

Answer 1

• Interstitial lung disease: DAD, sarcoidosis, CHP, IPF
• Pleura: effusion, pneumothorax, thickening
• Nerve/muscle: amyotrophic lateral sclerosis (motor neurone disease)
• Bone: Kyphscoliosis, ankylosing spondylitis, rib fractures
• Others: obesity, pregnancy

Question 2

Describe the investigation of sarcoidosis

Answer 2

• PFT, WBC, U&E, ECG, TB skin test/eye exam
• Bronchoscopy including transbronchial biopsy and EBUS
• CT
• Surgical biopsy maybe

Question 3

Describe the clinical management of sarcoidosis

Answer 3

• Mild: no treatment
• Arthralgia, erythema: NSAIDs
• Skin lesions, cough: topical steroids
• Cardiac, neurological, optic, hypercalcaemia: systemic steroids

Question 4

Describe the clinical management of idiopathic pulmonary fibrosis

Answer 4

• Median survival 3 years
• Refer to ILD clinic
• Palliative care
• Oral anti-fibrotic
• Perfinodone (pyridone)
• Nintedanib (kinase inhibitor)
• Lung transplant