Clinical Management and Features of Restrictive Lung Diseases

Question 1

Physiological definition of restrictive lung disease.

Answer 1

Physiological definition of restrictive lung disease - forced vital capacity <80% of normal

Question 2

Describe the different causes of restriction.

Answer 2

Lung causes of restriction - Interstitial lung diseases: idiopathic pulmonary fibrosis, sarcoidosis and hypersensitivity pneumonitis.

Pleural causes - pleural effusions, pneumothorax, pleural thickening.

Skeletal causes - kyphoscoliosis, ankylosing spondylitis, thoracoplasty, rib fractures.

Muscle causes - Amyotrophic lateral sclerosis

Sub-diaphragmatic causes - obesity and pregnancy.

Question 3

What are the common restrictive diseases.

Answer 3

Sarcoidosis, idiopathic pulmonary disease, hypersensitivity pneumonitis.

Question 4

Define sarcoidosis

Answer 4

Sarcoidosis - multisystem granuloma disease of unkown cause. Histological hallmark - non-caseating granuloma.

Question 5

Investigations for sarcoidosis.

Answer 5

Investigations - History and exam, CXR

CXR signs - bulky glands, hilar lymphadenopathy.

Other investigations - Pulmonary function tests, bloods/urinalysis,ECG/TB skin test/eye exam.

Further assessment - bronchoscopy including transbronchial biopsies and endobronchial ultrasoung.

Surgical biopsies - may be needed; mediastinoscopy, video assisted thoracoscopic lung biopsy.

Question 6

Describe remission rates for sarcoidosis.

Answer 6

Remission rates: Stage 1 - 55-90%, Stage 2 - 40-70%, Stage 3 - 10-20%, Stage 4 - 0%

Question 7

Describe treatment for sarcoidosis.

Answer 7

No treatment for mild disease with no vital organ involvement and normal lung function and few symptoms.

Sarcoidosis with erythema nodosum/antralgia treatment - NSAIDs.

Sarcoidosis with Skin leasions/anterior uveitis/ cough treatment - topical steroids.

Cardiac sarcoids, neurological, eye disease not responding to topical Rx, hypercalcaemia - systemic steroids.

Question 8

Describe presentation of idiopathic pulmonary disease.

Answer 8

Presentation of Idiopathic pulmonary fibrosis -
- chronic breathlessness and cough
- typically 60-70 years old
- commoner in men.
- Failed Rx for LVF or infection.
- Clubbed and crackles.

Question 9

Describe options to treat idiopathic pulmonary disease.

Answer 9

Options - 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.