Restrive Lung Disease Flashcards
What is the interstitium of the lung?
The space between the basement membranes of the alveolar walls
What happens during interstitial lung disease?
Pathological material is laid down in the interstitium which thickens the alveolar walls reducing gas exchange
What is the result of interstitial lung disease?
Reduced lung compliance Low FEV1 Low FVC FEV1/FVC ratio normal Reduced gas transfer V/Q imbalance
What are the possible presentations of interstitial lung disease?
Asymptomatic
Dyspnoea
Type 1 respiratory failure
Heart failure
What are the three types of chronic response to interstitial lung injury?
UIP
Granulomatous response
Other patterns
What can interstitial lung injury lead to?
End-stage honeycomb lung
What type of chronic response is most likely to develop into fibrosis?
Usual interstitial pneumonitis
What causes diffuse alveolar damage?
Major trauma Chemical injury Circulatory shock Drugs Infection Autoimmune disease Radiation
What are the histological features of DAD?
Protein rich oedema Fibrin Hyaline membranes Denuded basement membrane Epithelial proliferation Fibroblast proliferation Scarring of the interstium and the airspaces
What diseases come as part of the granulomatous response?
Sarcoidosis
Hypersensitivity pneumonitis
What is the most common interstitial lung disease?
Sarcoidosis
What are the histological features of sarcoidosis?
Epithelioid and giant cell granulomas
Necrosis/ caseation very unusual
Little lymphoid infiltrate
Variable associated fibrosis
What are the most common sites of sarcoidosis?
Lungs
Intrathoracic lymph nodes
What is the acute presentation of hypersensitivity pneumonitis?
Fever Dry cough Myalgia Chills Crackles Tachypnoea Wheeze Precipitating antibody
What is the chronic presentation of hypersensitivity pneumonitis?
Insidious Malaise SOB Cough Low grade illness Crackles and some wheeze
What are the histological features of hypersensitivity pneumonitis?
Soft centriacinar epithelioid granulomata
Interstitial pneumonitis
Foamy histiocytes
Bronchiolitis obliterans
What is hypersensitivity pneumonitis?
An immune complex mediated combined Type 3 and Type 4 hypersensitivity reaction
What are the possible causes of UIPs?
Connective tissue disease Drug reaction Post infection Industrial exposure Idiopathic
What are the histological features of UIP?
Patchy interstitial chronic inflammation
Type II pneumocyte hyperplasia
Smooth muscle and vascular proliferation
Proliferating fibroblastic foci
What are the signs and symptoms of UIP?
Dyspnoea Cough Basal crackles Cyanosis Clubbing
