Clinical Features and management of Restrictive Lung Diseases

Question 1

What spirometry reading is characteristic of restrictive lung disease

Answer 1

FVC of <80%

Question 2

List the different causes of restrictive lung diseases

Answer 2

Interstitial lung disease (idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis)

Pleural causes (effusion, pneumothorax, pleural thickening)

Musculoskeletal causes (kyphoscoliosis, ankylosing spondylitis, thoracoplasty, rib fractures, amylotrophic lateral sclerosis)

Other causes (obesity and pregnancy)

Question 3

What are interstitial lung diseases?

Answer 3

Interstitial lung disease is an umbrella term which encompasses >200 diseases all of which cause thickening of the Interstitium and can result in pulmonary fibrosis

Question 4

What are the top 3 interstitial lung diseases?

Answer 4

1. Sarcoidosis
2. Idiopathic pulmonary fibrosis
3. Hypersensitivity pneumonitis- Caused by natural allergens

Question 5

Define sarcoidosis

Answer 5

a multisystem granulomatous disease of unknown cause

Question 6

What is the histological hallmark of sarcoidosis?

Answer 6

non-caseating granuloma

Question 7

How do sarcoidosis patients present?

Answer 7

erythema nodosum, ocular redness, skin lesions, lupus perino (nasal lesion) and localised inflammation of scar tissue

Question 8

What is the epidemiology of sarcoidosis?

Answer 8

• Adults<40

* Women>men

Question 9

How should sarcoidosis be investigated?

Answer 9

1. Clinical history and examination
2. CXR
3. Pulmonary function tests, bloods, urinalysis, ECG, TB skin test & eye exams should be carried out to eliminate the differentials (including TB)
4. Tissue diagnosis

Question 10

What would you expect to see on a CXR of a patient with sarcoidosis?

Answer 10

hilar lymphadenopathy

paratracheal enlargement.

“Spotty lungs”

interstitial damage & fibrosis

Question 11

What is the treatment for mild, mild/moderate, moderate/severe and severe sarcoidosis?

Answer 11

mild- No treatment
mild/moderate- NSAIDS
moderate/severe - topical steroids
severe- topical steroids

Question 12

What are the differing clinical signs associated with mild, mild/moderate, moderate/severe and severe sarcoidosis?

Answer 12

mild- few symptoms

mild/moderate- Erythema Nodosum/ arthralgia

moderate/severe - Skin lesions/ anterior uveitis/ cough

severe- Cardiac or neurological involvement/ eye disease not responding to topical Rx/ hypercalcaemia

Question 13

How does idiopathic pulmonary fibrosis present as clinically?

Answer 13

Chronic breathlessness and cough

Failed to respond to previous prescriptions for Left ventricular failure or infection

Clubbed nails and crackles (which won’t improve with antibiotics or diuretics)

Shadowing in the lung bases on CXR (caused by fibrosis, not effusion) and bronchiectasis on CT

Question 14

What is the median survival of idiopathic pulmonary fibrosis?

Answer 14

3 years

Question 15

How should idiopathic pulmonary fibrosis be managed?

Answer 15

• Refer the patient to an ILD clinic
• Oral anti fibrotic to stop the disease getting worse
• Transplant is the only surgical option
• Palliate

Question 16

How is idiopathic pulmonary fibrosis distinguished from sarcoidosis?

Answer 16

Idiopathic pulmonary fibrosis presents as a fibroblastic focus made up of collagen (rather than a sarcoid lesion which is granulatomas as in sarcoidosis)

Question 17

Describe a typical idiopathic pulmonary fibrosis patient

Answer 17

60-70 year old male