Restrictive Lung Disease

Question 1

What is the interstitium of the lungs?

Answer 1

Connective tissue space bound airways and vessels and the space between the basement membranes of the alveolar walls

Question 2

What happens to the alveolar walls in disease?

Answer 2

Wall is thickened by interstitial infiltrate, therefore there is more distance for the gas molecules to cross making gas exchange more difficult

Question 3

What are the presentations of diffuse lung disease?

Answer 3

Anormal CXR, when patient is still asymptomatic - usually taken for another reason
Dyspnoea at rest and on exertion
Type 1 respiratory failure
Heart failure

Question 4

What are the causes of diffuse lung disease?

Answer 4

Parenchymal injury:
Acute response - diffuse alveolar damage
Chronic response - Usual interstitial pneumonitis is the pathological process, Granulomatous responses,
Leads to fibrosis

Question 5

What is diffuse alveolar damage associated with?

Answer 5

Major trauma
Chemical injury/toxic shock
Circulatory shock
Drugs
Infection
Autoimmune disease
Radiation

Question 6

What are the histological features of diffuse alveolar damage?

Answer 6

Protein rich oedema
Fibrin
Hyaline membranes
Denuded basement membranes
Epithelial proliferation
Fibroblast proliferation
Scarring

Question 7

What is sarcoidosis?

Answer 7

A multisystem granulomatous disorder of unknown aetiology

Question 8

What is the histopathology of sarcoidosis?

Answer 8

Epithelioid and giant cell granulomas

Variable associated fibrosis

Question 9

What are the commonly involved organs in sarcoidosis?

Answer 9

Lymph nodes, skin, lung, spleen, liver, bone marrow, eyes

Question 10

What are the common presentations?

Answer 10

In young adults: Acute arthralgia, erythema nodosum and bilateral hilar lymphadenopathy
Incidental abnormal CXR
SOB, cough

Question 11

What is involved in the diagnosis of sarcoidosis?

Answer 11

Clinical findings
Imaging findings
Serum calcium and ACE
Biopsy

Question 12

What are the antigens involved in hypersensitivity pneumonitis?

Answer 12

Thermophilic actinomycetes
Bird/animal proteins
Fungi
Chemicals

Question 13

What are the acute presentations of hypersensitivity pneumonitis?

Answer 13

Fever, dry cough, myalgia
Chill 4-9 hours after AG exposure
Crackles, tachypnoea, wheeze
Precipitating antibody

Question 14

What are the chronic presentations of hypersensitivity pneumonitis?

Answer 14

Insidious
Malaise, SOB, cough
Low grade illness
Crackles and some wheeze

Question 15

What is the histopathology of hypersensitivity pneumonitis?

Answer 15

Immune complex mediated combined type III and IV hypersensitivity reaction
Soft centriacinar epitheloid
Interstitial pneumonitis 
Foamy histiocytes
Bronchiolitis obliterans 
Upper zone disease

Question 16

What is the histopathology of UI{P?

Answer 16

Patchy interstitial chronic inflammation
Type II pneumocytes hyperplasia
Smooth muscle and vascular proliferation 
Evidence of old and recent injury
Proliferating fibroblastic foci