Restrictive Lung Disease Flashcards
What is the definition of restrictive physiology in regards to lung volumes?
Lung volumes are reduced
decreased TLC, FRC, FEV1 and FVC
What type of work is affected by obstructive disease and during which phase of respiration?
increase in resistive work in both inspiration and expiration
What type of work does restrictive lung dz affect and during which phase?
increase in elastic work and affects inhalation ONLY
what is the composition of work of breathing?
elastic and resistive work
What does a decrease in compliance cause in elastic work/
increase in elastic work
What 3 factors affect lung compliance?
- tissue content of the lung
- lung H20
- surface tension
What happens to lung compliance with increased lung h20 and decreased surfactant?
decreased lung compliance
What 2 features distinguish restrictive lung dz from restrictive physiology?
- Slope of the PV curve is reduced in restrictive dz
2. DLCO/VA is decreased in restrictive dz
what is the difference btw restrictive lung dz and restrictive physiology?
restrictive lung dz is if you have a dz in your lung whereas restrictive physiology is if you are fat or have a neuromuscluar problem
True or False: Chest wall disease changes the slope of the PV curve
False, PV curve has the same slope as normal
T/F Restrictive chest wall disease causes gas exchange problems
False: gas exchange unit is fine its just the chest wall that is screwed up
What is the first abnormality seen in patients with pulmonary fibrosis?
reduced DLCO
What 2 criteria must be made to have mixed obstructive and restrictive dz?
- reduced lung volumes
2. FEV1/FEV
what is the most common cause of interstitial lung disease?
autoimmune
what is the most important factor for figuring out the type of interstitial lung disease?
underlying etiology (history)
What 2 things must you combine to make an accurate diagnosis of ILD?
- etiology
2. pathology
what are common findings for physical exam of ILD?
auscultation: end-inspiratory “velcro like” crackles
distal clubbing in advanced cases
joint an skin can elude to connective tissue disease
What is the pathology for idiopathic pulmonary fibrosis?
areas of normal tissue next to areas of fibrotic tissue. Heterogenous involvement and different stages of scarring and honeycombing
what does an X-ray look like for IPF?
peripheral and basilar predominate reticulation, honeycombing, and ground glass
what is an alveolar wall finding that is distinctive feature of Non-specific interstitial pneumonia (NSIP)?
alveolar wall thickening
what is a radiographic finding of NSIP?
Diffuse fibrosis
what is organizing pneumonia?
non-infectious pneumonia
what is the treatment for organizing pneumonia?
steroids but they are required for 6-12 months
what is the pathologic findings of organizing pneumonia?
plugs of granulation tissue and fibrosis distal to bronchioles
how do you diagnose acute eosinophilic pneumonia?
> 20% eosinophils
how do you treat eosinophilic pneumonia?
steroids
what’s the radiographic finding of chronic eosinophilic pneumonia?
photographic negative of pulmonary edema
what patient population does lymphangioleiomyomatosis (LAM)?
cystic lung disease that only affects young women
what mutation causes LAM?
mutation in tuberous sclerosis gene that causes peribronchovascular proliferation of smooth muscle
what is the common radiographic finding on LAM?
cysts that are distributed profusely
how do you treat LAM?
mTOR inhibitors
what is a common site of cancer in LAM?
angiomyolipomas (renal tumor)
what is the characteristic pathology of sarcoidosis?
non-caveating granulomas
What type of pattern of disease do sarcoid patients show/
restrictive, obstructive, or mixed PFT
What is the treatment for sarcoidosis?
no tx necessary however can use steroids or cytotoxic drugs
what is fibrous plaques consistent with?
usual interstitial pneumonia
