Pulmonary Fibrosis Flashcards
Shared ILD features
Diffuse, bilateral pulmonary involvement (patchy?)
Distortion or destruction of alvoeli associated with inflamm cells
IPF key things
Older adults, limited to lungs, cannot explain why
Risk factors of IPF
Age Cigarettes Environmental Fam history GE rflux Viruses (EBV other herpesviruses)
Major dx criteria
Exlusion of others
Abnormal pulm function studies show evidence of restriction and impaired gas exchange
Compatible HRCT
Transbronchial lung biopsy excluding other d
Minor dx
Age over 50
Insidious onset of unexplained dyspnea
Sx over 3 mos
Bibasilar inspiratory crakcles
Need all 4 major plus 3 minor to dx
Clinical evals of pts with dyspnea
Spirometry, lung volumes, diff capacity, exercise study…measure O2 sat with and without exercise
CXR and HRCT
Labratory data
Pulm function studies
LOw VC and TLC
FEV/FVC >75%
Reduced compliance
<60% predicted diffusion capacity
INcreased alv-arterial O2 difference (determined by alveolar air equation)…or resting or exercise hypoxemia
If coexisting with emphysema, could alter these
Gross pathology
Cobblestone lung
Lab studies to do
CBC, sed rate, liver, urinalysis
HS pnuemonitis hx
Think bird cages
Pathogenesis
Patchy, progressive epithelial injury and disruption of alveolar basement membranes
Ineffect reapir
Alvolear fibrosis associated with fibroblastic foci at the lung periphery
Keloid formation
Molecular endotyping
INjured epithelial cells basically recruit more fibroblasts
Fibroblastic focus
MUST be seen in order to call IPF
Start at the periphery and move inward
Once dx made
Immunize, O2, talk about end of life planning
What happens to pts?
Rapid progression
Slow progression
Stable
Most will have an acute worsening (exacerbation)…suddenly decline and die
