Restrictive Lung Disease + IPF Flashcards
What restrictive lung disease?
Characterized by either a reduction in lung compliance or increased external pressures around the lungs, limiting lung inflation during inhalation
What are external etiologies of restrictive lung disease?
Fluid
Masses
Abnormal thoracic anatomy
What are intrinsic etiologies of restrictive lung disease?
Loss of lung compliance due to pathological lung parenchymal changes
-thickening or fibrosis of alveolar tissues
-lungs become stiff
What would a PFT of restrictive lung disease look like?
TLC decreased
FEVI decreased
FVC decreased
FEVI/FVC 2
- Normal: the FVC and FEVI are equally effected
- Increased: FVC reduction is larger than FEVI
What is acute restrictive lung disease?
It leads to sudden respiratory distress and potentially failure
Sudden influx of fluid into alveolar spaces and tissues (edema)
ARDS- acute respiratory distress syndrome.
Sudden, massive collection of fluid in the pleural space
Hemothorax or pleural effusion
What is chronic restrictive lung disease?
ILD Interstitial lung disease
What is idiopathic pulmonary fibrosis IPF?
It’s a type of IIP- idiopathic interstitial pneumonia
Restrictive lung disease that results in diffuse lung scarring (fibrosis)
IPF =
UIP Usual Interstitial pneumonia
How common is IPF?
The most common type of idiopathic interstitial pneumonia
Although idiopathic, what do we think is the pathology for IPF
Likely due to recurrent cycles of epithelial cell injury and dysregulated repair
What factors are associated with IPF?
Smoking
Autoimmune/rheumatic disease
Long term exposure to irritant fumes/dust particles
How does IPF present clinically?
Dry Cough
Progressive dyspnea
(TOTALLY ISOLATED TO LUNGS)
What kind of lab do you run to check for IPF?
Rhematological work up to rule out autoimmune etiology
What would you see in spirometry of IPF?
Restrictive
TLC Decreased
FEVI Decreased
FVC
FEVI/FVC Normal or increased
What would you see on chest x-ray of IPF?
Increased reticular markings
“Ground glass” opacities***
What would you see on chest CT scan of IPF?
Increased reticular opacities and honeycomb-type changes
Ground glass opacities
Do we do a lung biopsy on everyone?
It is the gold standard for definitive diagnosis but its only necessary for atypical cases.
How do you treat IPF?
Supplement O2
-Initially only with exertion but later on continuously
- helps slow the progression of PHTN
What medications can you give a patient with IPF?
Immune modulating medications being tested by FDA
-Nintendanib and pirfeniodone
What do immune modulating medications do in IPF?
Slows the progression of cellular fibrosis, does not reverse fibrosis.
What is the only definitive treatment for IPF?
Lung transplant
What is the survival rate with lung transplant?
50% 5 year survival rate
