interstitial lung dz Flashcards
ILD
Accumulation of inflammation in the interstitium; progressive scarring leads to lung/air sac stiffening and once this happens its irreversible
idiopathic chronic multisystemic inflammatory non-caseating granulomatous disease
aka– small patches of swollen tissue (granulomas) develop in organs
sarcoidosis
risk factors for sarcoidosis
black females
treatment for sarcoidosis
prednisone
sx of sarcoidosis
50% asymptomatic
* dry cough
* intrathoracic lymphadenopathy
* erythema nosoum or lupus pernio
* eyes involvement
most accurate way to diagnose sarcoidosis vs best initial test
tissue biopsy is most accurate
chest radiographs is best initial
finding of sarcoidosis on chest radiographs & PFT
bilateral hilar lymphadenopathy on radiographs
restrictive findings (normal or increased ratio)
- Chronic progressive fibrosing interstitial pneumonia of unknown etiology
idiopathic pulmonary fibrosis
risk factors for IPF
men over 50
cigarette smoking!!
- dry cough
- SOB then fatigue
- clubbing
- bibasilar end-inspiratory crackles/rales
pulmonary fibrosis sx
“reticular opacities” (honey combing) at lung bases should make you think of?
pulmonary fibrosis
life expectancy of IPF
3-4yrs
CT scan shoes usual interstitial pneumonia, reticular honeycombing, focal ground-glass opacification & surgical path/histo. shows UIP
IPF
tx of IPF (2)
- steroids for exacerbations (can worsen prognosis)
- lung transplant is only cure
- chronic fibrotic lung diseases secondary to inhalation of mineral dust
pneumoconiosis
4 pneumoconiosis talked about in lecture
asbestosis
silicosis
coal workers pneumo.
hypersentivity pneumonitis
which asbestos fiber is harder to remove, more toxic and reaches deeper into lungs
amphiboles
most common form of exposure to asbestos
general community exposre
other forms: work, bystander
asbestosis pathophys
- deposition & transmigration of the fibers
- macrophage accumulation then fibroblasts
- fibrosis increases over time
- functions as tumor initaiator and promoter
- smoking
- DOE
- nonproductive cough
- bibasilar end-inspiratory rales
- RHF if advanced
- clubbing
- reduced chest expansion
asbestosis
NOTE: sx similar to IPF, before imagings use history as guide!
PFT and ABG findings with asbestosis
- PFT– restrictive
- ABG may show resp. Alkalosis
CXR: diffuse reticonodular infiltrates at lung bases “shaggy heart borders”
CT: ground-glass, pleural thickening & calcified pleural plaques by border
asbestosis findings
3 major effects of asbestos exposure
- pleural and pulmonary fibrosis
- cancers of resp. tract
- mesothelioma
caused by inhaling silica dust
silicosis
caused by inhaling coal mine dust
coal workers pneumoconiosis/black lung dz
- eggshell calcifications on hilar & mediastinal nodes
- biopsy shows dust-laden macrophages & loose reticulin fibers
- non specific tx
- often asymptomatic but can have DOE, dry cough, rales, fatigue
silicosis
- chest radiograph: small nodules predominantly in upper lung w/ hyperinflationof lower lobes in obstructive pattern (resembles emphysema)
- biopsy (not needed) shows black lungs– interstitial pigment deposition and anthracitic macrophage
- tx includes pulm rehab
CWP/black lung
- Results from repeated inhalation of/sensitization to aerosolized organic antigens
hypersensitivity pneumonitis
- acute: rapid on set of flu like sxs, fever, chills, cough, dyspnea, myalgias 4-8 hrs after prolonged exposure to antigen
- chronic: DOE, cough, crackles, insp squeaks
- farmers lung from moldy hay
- restrictive component
- CT– upper lobe predominance, ground glass and fibrosis, honeycombing
hypersentivity pneumonitis
tx of hypersensitivity pneumonitis
avoid exposures
steroids
lung transplant
