Pathology of Restrictive Lung Disease Flashcards
What is the interstitium of the lung?
Connective tissue space around the airways and vessels and the space between the basement membranes of the alveolar walls
What is the connective tissue space around the airways and vessels called?
Interstitium of the lung
How are alveolar epithelial (pneumocytes) and interstitial capillary endothelial cell basement membranes in normal alveolar walls?
In direct contact
What does restrictive lung disease show?
Reduced lung compliance (stiff lungs)
Low FEV1 and low FVC but same FEV1/FVC ratio
Reduced gas transfer
Ventilation/perfusion mismatch
What does the spirometry of restrictive lung disease look like?

What is the presentation of restrictive lung disease?
Abnormal chest X-ray
Dyspnoea (on exertion and rest))
Respiratory failure (type 1)
Heart failure
What is dyspnoea?
Difficult breathing
What is difficult breathing called?
Dyspnoea
What is the difference in chest X-rays between:
Emphysema
Normal
Restrictive lung disease
Emphysema can see all ribs
Normal can see 10 ribs
Restrictive lung disease can see less than 10

What is restrictive lung disease also known as?
Interstitial lung disease
What is the pathway leading to restrictive lung disease?
1) Lung injury
2) Leads to chronic response
3) One of usual interstitial pneumonitis (UIP), granulomatous response, or other pattens leads to
4) Fibrosis or end stage honeycomb lung

What does the acute response to lung injury lead to?
Diffuse alveolar damage
What is diffuse alveolar damage associated with?
Major trauma
Chemical injury/toxic inhalation
Circulatory shock
Drugs
Infection
Autoimmune disease
Radiation
Idiopathic
What does idiopathic mean?
No known cause
What is no known cause called?
Idiopathic
What does the extend of diffuse alveolar damage depend on?
How many days the injury lasts for

What does DADS stand up for?
Diffuse alveolar damage
What are the histological features of acute alveolar damage?
Protein rich oedema
Fibrin
Hyaline membranes
Denuded basement membranes
Epithelial proliferation
Fibroblast proliferation
Scarring
Why do granulomatous responses to chronic inflammation lead to fibrosis or end stage honeycomb lung?
Sarcoidosis
Hypersensitivity pneumonitis
What is sarcoidosis?
Multisystem granulomatous disorder of unknown aetiology
What is a multisystem granulomatous disorder of known aetiology?
Sarcoidosis
What is the histopathology of sarcoidosis?
Epitheloid and giant cell granulomas
Necrosis/caseation very unusual
Little lymphoid infiltrate
Variable associated fibrosis
What is a granulomas?
Structure formed during inflammation that is found in many diseases, being a collection of macrophages
What is a structure formed during inflammation that is composed of a collection of macrophages?
Granulomas
