interstitial lung disease Flashcards
What is meant by Interstitial Lung DIsease?
chronic, heterogenous group of disorders that involve variable degrees of inflammation, infiltration, and fibrosis of alveolar-capillary units and adjacent interstitium
what is the typical lung presentation of ILD?
small non-compliant lungs with abnormal gas exchange
What are known causes of ILD?
pneumoconiosis (silicosis, asbestosis), toxins,drugs, radiation, oxygen, hypersensitivity
ILD can sometimes be caused by what class of diseases?
fibrotic lung disease associated with collagen vascular disease
(ex: rheumatoid arthritis, scleroderma, mixed CT disease, etc)
What is the most common of the idiopathic interstitial pneumonias?
Idiopathic Pulmonary Fibrosis-
where do we see ipf?
limited to LUNGS
What are the main clinical features of IPF?
dyspnea on exertion, paroxysmal noproductivecough, crackley breathsounds,tachypnea, clubbing, restrictivepulmonary physiology with reducedlungvolumes,DLCOand wide Aa difference
What are three main manifestations on high resolution CT scan in IPF?
irregular reticular densities, traction bronchiectasis (scarring pulls the bronchioles open), honeycomb-ing subpleural.
How is sarcoidosis different from IPF?
effects every part of the body. lots of granulomas. BIG lymph nodes. PFT show mixed obstructive and restrictive. GOOD prognosis
what is unique about the granulation tissue that forms in IPF?
prolif of fibroblasts and formation of myofibroblasts transformation of pneumocytes into myofibroblasts (epithelial-mesenchymal transition)
what up with desquamative interstitial pneumonitis?
Rare, linked to smoking, younger, response to steroids, good prognosis.
-lots of macrophages in alveoli, interstitial lymphocytes, MILD interstitial fibrosis
WHICH form of Nonspecific interstitial pneumonia has the better prognosis?
cellular form
Which IP has the most uniformpresentation offibrosis?
Non specific! homogenous, uniform fibrosis
what is boop?
bronchiolitis obliterans organizing pneumonia!
Granulation tissue plugs in alveoli/bronchi. lymphocytes fill the septa! giant cells present! can produce small airway obstruction
what types of hypersensitivity reactions are involved in hypersensitivity pneumonitis?
TYpes III and TYpe IV
