Interstitial lung disease - collins Flashcards
what are other names for interstitial lung disease
diffuse parenchymal disease
pulmonary fibrosis
what is interstitial lung disease
group of lung disorders that impair the interstitium - damage also extends to the parenchyma (capillaries, alveoli, bronchioles)
does not extend proximal to bronchioles
leads to impaired gas exchange
what is the pathophysiology of interstitial lung disease
inflammation -> fibrosis (deposits of connective tissue) -> impaired gas exchange
what are the most common types of interstitial lung disease
exposure related (drug and occupation/environmental)
autoimmune related
idiopathic (Most common)
how many types of interstitial lung disease are there
over 200 - we only talk about 3 major types
what are drug induced/exposure related interstitial lung disease
chemotherapy, methotrexate, amiodarone, macrobid
what are occupation/environmental related interstitial lung disease
inorganic or organic dust
gas/fumes
radiation
what are the autoimmune related interstitial lung disease
rheumatoid arthritis
SLE
Scleroderma
polymyositis
dermatomyositis
sjogrens syndrome
what are the different types of idiopathic interstitial lung disease
Idiopathic pulmonary fibrosis (IPF)
Sarcoid
acute interstitial pneumonia (AIP)
non-specific interstitial pneumonia (NSIP)
cryptogenic organizing pneumonia (COP)
what is the presentation of interstitial lung disease
often chronic symptoms (Months- years)
progressive exertional dyspnea m/c
may present as simple dry cough, SSX of autoimmune disease, asympathomatic (screening)
what are possible exam findings for interstitial lung disease
bibasilar crackles
clubbing of digits
PE consistent with pulmonary HTN
tachypnea
reduced chest expansion
what is the workup for interstitial lung disease
starts with thorough history
occupational history
thorough PMH with treatments: CT dz, malignancy, Rheumatologic condition
Family hx
lab tests to rule out other causes and check for known causes
what lab tests are typically run for interstistial lung disease
CBC, CMP, rheumatologic testing, HIV, HCV, etc
What are supportive CXR findings for interstitial lung disease
bronchiectasis 9Tram-track appearance)
miliary pattern
honeycombing
kerley lines
reticular or rediculonodual opacities
what are PFTs used for with interstitial lung disease
assess severity, prognosis and monitoring
what is a diagnostic test for ILD
biopsy
What are supportive CT findings for ILD
bronchiectasis
miliary pattern
honeycombing (coarse reticular)
kerley lines
reticular or rediculonobular opacities
what is the treatment for ILD
fibrosis is irreversible - goal is to prevent/limit further damage
no BEST treatment
coricosteroids (pred)
antifibrotic agent (pirfenidone and nintedanib)
treat GERD (iansoprazole, omeprazole, pantroprazole)
what are other supportive treatment options for ILD
supplemental O2
Pulmonary rehabilitation
Tobacco cessation
Flu and pneumococcal vaccines
lung transplant for refractory disease
what is the only definitive treatment for ILD
lung transplant for refractory
what is another name for idiopathic pulmonary fibrosis
usual interstitial pneumonia (histologic description)
what is the presentation of Idiopathic pulmonary fibrosis
DOE and dry cough, often chronic (1 year)
classiually with bibasilar end inspiratory crackles on exam
‘usual interntitail pneumonia pattern’ on imaging
what are usual interstitial pneumonia imaging findings
basilar reticular opacities
honeycombing
bronchiectasis
what are the easy diagnostic criteria
Age > 65
no known cause of SSx (idiopathic)
inspiratory crackles on PE
Restrictive PFT
Classic UIP imaging findings
