Restrictive Lung Diseases Flashcards
Interstitium of lung
The space between the basement membranes of the alveolar walls
Effects of restrictive lung disease
Reduced lung compliance
Low FEV1 and FVC but normal ratio
Reduced gas transfer
V/Q imbalance
Presentation of restrictive lung disease
Abnormal chest x-ray or CT scan
Shortness of breath on exertion and rest
Type 1 respiratory failure
Heart failure
X-ray of interstitial lung disease
Increase in lung markings and more shadowing between ribs
Types of responses
Acute response Chronic response Usual interstitial pneumonitis UIP Granulomatous responses Other patterns
End result of disease
Fibrosis or end-stage honeycomb lung
Acute responses
Diffuse alveolar damage (DAD)
Diffuse alveolar damage causes
Major trauma Chemical injury/toxic inhalation Circulatory shock Drugs Infection Autoimmune disease Radiation
Histological features of DADs
Protein rich oedema Fibrin Hyaline membranes Denuded basement membranes Epithelial and fibroblast proliferation Scarring - interstitium and airspaces
Granulomatous responses
Sarcoidosis Hypersensitivity pneumonitis (HP)
Sarcoidosis
A multisystem granulomatous disorder
Histopathology of sarcoidosis
Epithelioid and giant cells granulomas
Necrosis/caseation very unusual
Little lymphoid infiltrate
Variable associated fibrosis
Incidence of sarcoidosis in the UK
3-4/100,000
Most common organs involved in sarcoidosis
Lymph nodes
Lung
Spleen
Liver
Presentation of sarcoidosis 1
Acute arthralgia (joint pain) Erythema nodosum Bilateral hilar Lymphadenopathy
Presentation of sarcoidosis 2
Abnormal chest x-ray or CT - no symptoms
Presentation of sarcoidosis 3
Shortness of breath
Cough
Abnormal chest x-ray
Treatment of sarcoidosis
Corticosteroids
Diagnosing sarcoidosis dependant on
Clinical findings
Imaging findings
Serum calcium increased and ACE
Biopsy
Antigens of hypersensitivity pneumonitis
Thermophilic actinomycetes
Bird/animal proteins
Fungi
Chemicals
Acute presentation of Hypersensitivity pneumonitis
Fever Dry cough Myalgia Chills after Ag exposure Crackles Tachypnoea Wheeze Precipitating antibody
Chronic presentation of hypersensitivity pneumonitis
Insidious Malaise Shortness of breath Cough Low grade illness Crackles and wheeze More pulmonary damage
Histopathology of hypersensitivity pneumonitis
Immune complex mediated combined type III and IV hypersensitivity reaction
Soft centriacinar epithelioid granulomata
Interstitial pneumonitis
Foam histiocytes
Bronchiolitis obliterates
Upper zone disease
Usual interstitial pneumonitis (UIP) seen in
Connective tissue disease
Drug reaction
Post infection
Industrial exposure
2 main types of UIPs
Idiopathic pulmonary fibrosis (IPF)
Cryptogenic fibrosing alveolitis (CFA)
Histopathology of UIP
Patchy interstitial chronic inflammation Type II pneumocyte hyperplasia Smooth muscle and vascular proliferation Evidence of old and recent injury Proliferating fibroblastic foci
Idiopathic pulmonary fibrosis symptoms
Shortness of breath Cough Basal crackles Cyanosis Clubbing
Chest x-ray findings
Basal/posterior
Diffuse infiltrates
Cysts - honeycombing
‘Ground glass’
