Interstitial lung disease Flashcards
characterization of ILD
cellular infiltration
scarring
architectural disruption of parenchyma
ILD can compromise what locations of the lung?
alveolar wall
septum interstitium
lumina and walls of small airways
clinical presentation of ILD
acute, subacute or chronic progressive dyspnea on exertion dry cough restrictive PFTs CXR / CT - interstitial infiltrates with ground glass opacities
physical exam for ILD
clubbing erythema / nodosum / arthritis / rash tachypnea dry crackles / squeaks increased right heart pressures
what may be the earliest change in PFTs in the context of ILD?
decreased DLCO
what is the radiographic distribution of ILD?
upper / lower
central / peripheral
what is the granulomatous ILD?
sarcoidosis
which idiopathic interstitial pneumonitis (IIP) has the worst prognosis?
idiopathic pulmonary fibrosis (IPF)
what are the radiographic characteristics of IPF?
- lower lobes and peripheral distribution of reticular infiltrates
- fibrotic changes
what is the clinical use for nintedanib?
IPF
what are two medications for IPF?
nintedanib
pirfenidone
what is the clinical use for pirfenidone?
IPF
what is the radiographic presentation of nonspecific interstitial pneumonitis (NSIP)?
ground glass opacity symmetrically in the lower lung zones, with or without reticular infiltrates
DIP
desquamative interstitial pneumonitis
RB-ILD
respiratory bronchiolitis associated ILD
DIP and RB-ILD are associated with what activity?
smoking
AIP
acute interstitial pneumonitis
DAD
diffuse alveolar damage
what is the radiographic presentation of AIP / DAD?
diffuse, bilateral air space and reticular infiltrates
what is the problem associated with AIP / DAD? what is the treatment?
oygenation
supportive
COP
cryptogenic organizing pneumonia
patients with COP present with what type of symptoms?
pneumonia
what is the treatment for COP? what is the prognosis?
corticosteroids
good
what is the radiographic presentation for COP?
patchy infiltrates, unilateral or bilateral
50% small nodular opacities
consolidation and air bronchogram seen on HRCT
subpleural and peribronchiolar distribution
what is the mechanism for hypersensitivity pneumonitis?
cell mediated immunity - activation of alveolar macrophages and CD8 influx with ill defined granuloma formation
what is the acute presentation of hypersensitivity pneumonitis? (onset, PE, imaging)
abrupt onset of flu like symptoms
tachypnea and diffuse fine crackles
radiograph - normal or patchy, micronodular opacities
how does chronic presentation of hypersensitivity pneumonitis differ from acute?
digital clubbing
irreversible respiratory findings - FIBROSIS
what drug can cause commonly cause drug induced ILD?
amiodarone
what are highly suggestive radiographic findings for HP (hypersensitivity pneumonitis)?
ground glass opacification and centrilobular nodules
what are the radiographic findings of amiodarone toxicity?
ground glass and reticular opacities
increased attenuation liver and spleen seen on abdominal cuts
what are the two presentations for nitrofurantoin toxicity? what are the symptoms?
acute and chronic onset
acute - peripheral blood eosinophilia
chronic - increased serum Ig, serum transaminases, ESR, ANA+
what is the radiographic presentation of acute eosinophilic pnuemonia?
bilateral patchy ground glass and/or reticular opacities
increased eosinophilic count (over 25%) - key
what is the radiographic presentation of chronic eosinophilic pnuemonia?
bilateral peripheral or pleural based opacities described as “photographic negative” of pulmonary edema
subpleural consolidations, predominantly upper lobes
what are the features of lymphangioleiomyomatosis?
progressive cystic lung disease women of childbearing age associated with tuberous sclerosis proliferation of SMCs dyspnea on exertion, cough, hemoptysis
what is the radiographic presentation of lymphangioleiomyomatosis?
diffusely distributed thin walled cysts
what is the evaluation of ILD following history and PE?
chest radiography
blood tests
PFTs