Restrictive - Usual Interstitial Pneumonitis

Question 1

What 2 types of UIP is there?

Answer 1

Cryptogenic Fibrosing Alveolitis

Idiopathic pulmonary fibrosis

Question 2

Causes of UIP?

Answer 2

CT diseases
Drug reactions
Post infections
Industrial exposures

Question 3

Histopathology of UIP?

Answer 3

Interstitial inflammation

Type 2 pneumocyte hyperplasia

Proliferation of smooth and vascular muscle and fibroblastic foci

Question 4

What age group does it commonly affect?

Answer 4

Elderly

Question 5

Symptoms?

Answer 5

Clincally slow

Dyspnoea and cough

Question 6

Signs?

Answer 6

Basal crepitations
Cyanosis
Clubbing

Question 7

How progressive is the disease?

Answer 7

Very - most likely dead in 5 years

Question 8

What investigations can be done?

Answer 8

CxR

Pulmonary function tests

Question 9

What do CxRs see?

Answer 9

In basal area:

Infiltrates
Cysts
“Ground glass” opacification on lungs

Question 10

Management?

Answer 10

Send to an interstitial lung disease clinic
Oral anti-fibrotics
Some steroid response
Transplants

Poor prognosis overall