Interstitial Lung Disease Flashcards
(35 cards)
interstitial lung disease
aka diffuse parenchymal lung disease
disease of the alveolar wall, including the interstitium, the potential space between the basement membranes of the alveolar epithelium and the capillary endothelium
may also involve the alvolar space and small airways
four categories of ILDs
ILDs of known cause or association
idiopathic interstitial pneumonias
granulomatous ILDs
other (rare) DPLDs
interstitial lung diseases with a known cause
pneumoconiosis - exposure to inorganic dusts
hypersensitivity pneumonitis - immunologic response to inhaled organic dusts
reactions to chemicals, drugs, radiation, and toxic gases
those associated with connective tissue disease
proposed pathogenesis of ILD
age related susceptible lung
wound clot
aberrant epithelial cell activation
fibroblast focus
epithelial-mesenchymal transition
hypercoagulable milieu
exaggerated ECM accumulation
progressive lung remodeling
honeycomb changes

categories of idiopathic interstitial pneumonias
chronic fibrosing IIP
smoking-related IIP
acute/subacute IIP
chornic fibrosing IIPs
idiopathic pulmonary fibrosis (IPF)
idiopathic nonspecific interstitial pneumonia (NSIP)
smoking-related IIPs
respiratory bronchiolitis - interstitial lung disease (RB-ILD)
desquamative interstitial pneumonia (DIP)
acute/subacute IIPs
cryptogenic organizing pneumonia (COP
acute interstitial pneumonia (AIP)
symptoms of ILD
exertional dyspnea, usually insiduous
cough, usually non-productive
physical findings of ILD
fine, end-inspiratory crackles (“Velcro rales)
digital clubbing (IPF)
cyanosis
signs of cor pulmonale increased P2, RV lift, R-sided S3, JVD, edema (advanced disease)
findings on ILD chest x-ray
10% are normal at initial presentation
reticular, nodular, or reticulo-nodular interstitial pattern
diffuse and patchy alveolar pattern
reticular pattern
indicative of IPF

nodular pattern
indicative of sarcoidosis

alveolar pattern
much more homogeneous and confluent

common abnormalities of IL on HRCT
reticular opacities
nodular opacities
ground glass opacities
traction bronchiectasis
honeycomb cysts
traction bronchiectasis
bronchi are bigger than expected for specific lung sizes due to airways being pulled open

reticular opacities
indicative of scleroderma

nodular opacities
suggestive of sarcoidosis

ground glass opacities
suggests hypersensitivity pneumonitis

honeycomb cysts
suggestive of IPF

pulmonary function test results for ILD
restrictive defect most common
decreased lung volumes (TLC, FRC, RV)
normal or elevated FEV1/FVC
decreased lung compliance
reduced diffusion capacity
mixed restrictive and obstructive defect possible
arterial blood gas for ILD
increased A-a gradient, decreased PaO2
pneumoconiosis
ILD caused by inorganic dusts
silica -> silicosis
coal dust -> coal workers pneumoconiosis
asbestos -> asbestosis
hard metal alloys -> giant cell pneumonitis
berylium 0> berylliosis
hypersensitivity pneumonitis (HP)
ILD caused by inhalation of organic dusts
moldy hay -> farmer’s lung
bird proteins -> bird fancier’s disease


