Restrictive (interstitial) Lung Diseases Flashcards
What is the lung interstitium
The connective tissue space around the airways and vessels and is between the basement membranes of the alveolar wall
Features of Restrictive Diffuse Interstitial Lung Disease (4)
Reduced lung compliance
Low FEV1 and Low FVC but ratio is normal
Reduced gas transfer
Ventilation/Perfusion imbalance
Presentation of Diffuse Lung Disease (4)
Abnormal CXR
SOB on rest and exertion
Type 1 respiratory failure
Heart failure
Chronic Response (3)
Usual interstitial pneumonitis
Granulomatous
Fibrosis
Diffuse Alveolar Damage is Associated with (8)
Major Trauma Chemical injury.toxic inhalation Circulatory shock Drugs Infection Autoimmune disease Radiation Idiopathic
Evolution of DAD (4)
Exudate stage
Oedema and hyaline membrane thickening
Proliferative stage
Interstitial inflammation and fibrosis
Histological features of DAD (7)
Protein rich oedema Fibrin Hyaline membranes Denuded BM Epithelial proliferation Fibroblast proliferation Scarring of interstitium and airspaces
What is Sarcoidosis
A multisystem granulomatous disorder of unknown aetiology
Histopathology of Sarcoidosis (4)
Epitheloid and giant cell granulomas
Necrosis/caseation very unusual
Little lymphoid infiltrate
Variable associated fibrosis
Who does Sarcoidosis normally effect (3)
Young people
F>M
People in temperature climates
Presentation of Sarcoidosis
Acute arthralgia Erythema nodosum Bilateral hilar lymphadenopathy SOB Cough
Diagnosis of Sarcoidosis
Clinical findings Imagine findings Serum Ca++ and ACE Bx Kveim test Tuberculin test
Causes of Hypersensitivity Pneumonitis (4)
Thermophilic actinomycetes
Bird/Animal proteins- faeces, bloom
Fungi
Chemicals
Thermophilic actinomycetes (2)
Micropolyspora faeni
Thermoactinmycetes vulgaris
Acute presentation of hypersensitivity pneumonitis (7)
Fever Dry cough Myalgia Chills (4-9 hours) Crackles, tachypnoea Wheeze Precipitating Ab
Chronic presentation of hypersensitivity pneumonitis (6)
Insidious Malaise SOB Cough Low grade illness Can lead to respiratory failure
Immune complex in Hypersensitivity Pneumonitis
Type III and Type IV
Histopathology of Hypersensitivity Pneumonitis (6)
Immune complex Soft centriacinar epithelioid granuloma Interstitial pneumonitis Foamy histiocytes Bronchiolitis obliterans Upper zone disease
Causes of Usual Interstitial Pneumonitis
Scleroderma Rheumatoid disease Drug reaction Post infection Asbestos exposure Most are cryptogenic or Idiopathic
Histopathology of UIP (6)
Patchy interstitial chronic inflammation TYpe II pneumocyte hyperplasia Smooth muscle and vascular proliferation Temporal heterogeneity Spatial heterogeneity Proliferating Fibroblastic Foci
Most common individuals who get Idiopathic Pulmonary Fibrosis
Elderly >50
M>F
Clinical features of Idiopathic Pulmonary Fibrosis
Dyspnoea Cough Basal cracks Cyanosis Clubbing
CXR of Idiopathic Pulmonary Fibrosis
Basal/Posterior
Diffuse Infiltrates
Cysts
Ground glass
How can you correct Hypoxaemia caused by low V/Q
Increase FIO2
CO2 diffuses _____ faster than O2 because
20 times
it has greater solubility
What is a shunt
Blood passing from Right to Left side of Heart without contacting ventilated alveoli
What do large shunts respond poorly yo
Increase in FIo2