Restrictive Lung Disease - Path, Gupta Flashcards
definition of restrictive lung dz - signs, symptoms
heterogenous group of dz characterized by dyspnea and reduced TLC
what is the buzzword for the appearance of end stage lung dz in restrictive dz
honeycomb
what disease is a result of repeated cycles of injury caused by a yet unidentified agent resulting in diffuse fibrosis?
Idiopathic Pulmonary Fibrosis
presentation of idiopathic pulmonary fibrosis
insidious, DOE, dry cough
gradualy deteriorating course
treatment for IPF?
none current
how do you diagnose IPF?
by exclusion - you must rule out secondary causes of fibrosis such as drugs and radiation
what is the prognosis of IPF?
bad
in IPF, what is the appearance of the fibrosis? where is it found?
patchy interstitial fibrosis
predominantly lower lobe, subpleural distribution
will have areas of normal lung tissue intervening
**It is heterogenous in time and space - different areas of damage are at a different stage in the dz process than other areas
what are some key microscopic findings in IPF
fibroblast foci, collagenized areas, mild lymphocytic inflammation
what is the pathogenesis of IPF? - what is the key player in fibrosis
TGF-beta - from injured penumocytes induces fibrosis
in IPF, where is the concentration of fibrosis the highest - what is its distribution?
subpleural - towards periphery
what happens to the epithelium of bronchi in IPF
metaplastic change - goes from more PSSCE to squamous epithelium
for IPF, when you look at it histologically, what is it called?
usual interstitial pneumonia (UIP)
if you see an intraalveolar pattern of fibrosing, rather than interstitial, what dz are you thinking?
cryptogenic organizing pneumonia
in cryptogenic organizing pneumonia, compare the age of the various areas of organizing connective tissue
all of the same age (diff from IPF)
what is the treatment for cryptogenic organizing pneumonia
steroids
what must you differentiate cryptogenic organizing pneumonia from? (i.e. what dz process has a similar histologic pattern)
resolving acute lung injury/ infection - like pneumonia, or ARDS
what is a defining histologic finding in cryptogenic organzing pneumonia?
intraalveolar plugs of loose fibroconnective tissue
what is the name of a non-neoplastic lung reaction to inhaled dust, chronic exposure
pneumoconioses
what are 4 examples of dusts that can cause pneumoconioses
coal
silica
asbestos
beryllium
define anthracosis
what dz process do you see them in
Innocuous inhaled carbonaceous pigment engulfed by macrophages that accuulates in connective tissue along lymphatics and in lymphoid tissue
seen in Coal Workers Pneumoconiosis
in simple CWP, what is the pulmonary function? where do you find it in the lung?
little/no pulmonary dysfunction
upper lobe predominant
what is the pathogenesis of complicated CWP, and what is the lung function?
there is a progression from simple to complicated CWP over many years
leads to progressive massive fibrosis
will compromise lung function
what is histologic finding of complicated CWP
black scars composed of pigment and dense collagen
what is a high yield association for complicated CWP - another dz
rheumatoid arthritis
what is epidemiology associations for silicosis (pneumoconiosis caused by inhalation of silica)
sandblasters, mine workers, stone cutters
what is the histologic pathology of silicosis, and where in lung do you find it?
nodular fibrosis, mostly in upper lobes and hilar nodes
what is the specific pathophys of silicosis - i.e. what causes the fibrosis
silica ingestion by macrophages causes release of fibrogenic mediators - results in hard collagenous nodules
what is a diagnostic technique you can use to visualize silica in silicosis
polarizing microscopy demonstrates birefringent silica particles
what does silicosis increase your susceptibility to and why
TB
likely related to impaired function of macrophages
for pneumoconiosis due to Beryllium (Berylliosis?) - what is the histopathologic finding and where in what tissues do you see it
noncaseating granulomas
seen in lung, hilar lymph nodes and systemic organs
what does berylliosis leave you at increased risk for
lung cancer
taking a good pt history is necessary to differentiate what disease from berylliosis?
sarcoidosis
also has noncaseating granulomas in lung and systemic organs
(look at question stem for epidemiology shit)