Pulmonology Quiz 2b

eem8u's version from 2016-11-22 00:53


Question Answer
#1 risk factorcigarette smoking, 3rd ranked cause of death in US
COPD definitionpersistent EXPIRATORY airflow limitation that is 1) not fully reversible 2) assc w/ enhanced chronic inflammatory response in airways
****FEV1/FVC ratio (and w/ bronchodilator?)LOW <0.7 (remains low after bronchodilators
dynamic hyperinflation and exercise in COPDincrease in RR - decreased amount of time for expiration >> more volume left in lung (b/c of expiratory obstruction)
V/Q in chronic bronchitis vs emphysemalow v/q vs no mismatch b/c emphysema destroys entire unit
calculate DLcoDeco = (d-co X A) / T (diffusion constant of CO times surface area / thickness)
***DLCO in “pure” chronic bronchitis vs emphysemashould be normal vs low in emphysema (decrease Area)
pursed lips assc w/emphysema (create back pressure to prevent airway collapse)
rhonchi assc w/chronic bronchitis (gurgling from profuse secretions
hypoxia and INFECTIOn more assc w/chronic bronchitis “blue bloaters”


Chronic Bronchitis
Question Answer
diagnostic criteriaat least 3 months of productive cough, at least 2 years (w/ periods of exacerbations)
definedHYPERSECRETORY process - pathologic changes in mucus and mucus-secreting apparatus
***assc w/ what inflammatory cellNEUTROPHILS (not eo’s)
sequelae of smoking >> COPD (3)1- impair mucociliary clearance 2- bronchial wall inflammation >>damage epithelium 3- mucus gland hypertrophy/hyperplasia (so much junk coming in!
****Reid Index- define and chronic bronchitis vs normalratio of thickness of mucus gland layer/thickness of bronchial wall between EPITHELIUM and CARTILAGE (perichondrium) — (normal .4 // enlarge = >.5 as in chronic bronchitis)
****pneumothorax assc w/bullous emphysema, paraseptal/distal emphysema


Question Answer
Emphysema defineddestruction of lung tissue (esp elastic component ) + enlargement of air spaces DISTAL to terminal bronchiole (WITHOUT FIBROSIS) >> diminished expiratory drive and loss of tethering
centrilobular emphysema affects _________(part)___ and most commonly in _________(lung)______ zonePROXIMAL acinus (where smoke hits first), UPPER Lung - APEX (smoke rises in lung) // SMOKING **95% of cases ** (or coal dust)
***panlobular emphysema affects ____ and is most commonly in _____ zone // assc w/UNIFORM and diffuse involvement of acinus (but not entire lung) // LOWER zones // A1AT deficiency!
***paraseptal (distal acinar) emphysema affects ____ and is most commonly in _____ zone // assc w/distal acini (more adjacent to pleura at margins of lobules / UPPER HALF of lungs / young adults, with result of pneumothorax


Question Answer
Name the components of the ACINUSacinus = terminal bronchiole + respiratory bronchiole + alveolar duct + alveolus
***Calculate PalvPalv = Ppl + PL
PL and Palv in emphysemaLower because decreased elastic pressure
explain equal pressure pointwhen positive pressure during exhalation = Patm (this happens more in proximal airways in emphysema and can cause COLLAPSE due to no cartilage or tethering) // higher the initial P of expiration — further down the EPP
VC and RV changeshigher RV (and FRC and TLC) / lower VC
death results fromrespiratory acidosis and coma
bullous emphysema, definedany form that produces blebs or bulla > 2 cm —> often sub pleural, near apex, may rupture >>> penuothorax/hemorrhage


Question Answer
role of a1 anititrypsininhibitor of neutrophil elastase (proteolytic enzyme released by neutrophils and M-phages) - AAT provides 90% of inhibition in plasma
(2) roles of smoking in protease-antiprotenase balance1) recruits neutrophils (release elastase) 2) inactivate A1AT >> emphysema
***Z variant mutationglu342 >lys342
***why does z variant cause liver damagelys342 variant accumulates in hepatocyte ER >> cirrhosis
MZ vs ZZ phenotypesreduced a1at functional levels vs. DEFICIENCY


Question Answer
proven survival benefit (2)smoking cessation / supplemental o2 for hypoxemia
symptom management(1) bronchodilators (b2 agonists, anticholinergics, methylxanthines) /// (2) a1at protein infusion (3) lung volume reduction (4) lung transplant
lung volume reduction - what conditions? 3 physiologic benefitEMPHYSEMA — 1) improves elastic recoil 2) decrease dead-space volume 3) improves mechanical fx of diaphragm

Occupational Lung Disease

Question Answer
• impaction - where assc w/ what particle size>20 um (and much > 5) — trapped in turns of nasal turbinates
sedimentation - where assc w/ what particle size1-5 um (settles because of weight) -trapped in in small airways
diffusion - where assc w/ what particle sizeparticles < 01. um diameter — small airways and alveoli
silicosis requires at least ___ years of exposure20 years
pathogenesis of silicosisparticles (esp fresh cut) in lower resp >> phagocytksed mphage >> inflammatory mediator release & apoptosis >> release of toxic silica >> alveoli’s & FIBROSIS (silica noodle)
simple vs complicated silicosissmall rounded nodules vs MASSIVE fibrosis
silicosis affects which lung zoneUPPER
susceptibility to mycobacteria w/silicosis > impaired Mphage fx
coal worker’s pneumoconiosis - CWP pathogenesismassive amount of dust >> respiratory bronchioles >> focal dust emphysema (fibrogenesi w/ when admixed with silica)
***coal macule vs nodulemacule- aggregation of dust and mphage v nodule- dust/mphage/collagen (result from coal mixed with silica)


Question Answer
assc/ w very few clinical symptomssimple coal worker’s pneumonia
risk factor for complicated CWPsilica exposure
radiographic fea. of complicated CWPcoalesced opacities (calcified or caveated)
asbestosis usually assc _____ years exposure10-20 years
fibrosis in asbestos exposure occurs whendust load high/ incomplete phagocytes of fibers >> profinflammation and cytotoxic elements >> fibroblast recruitment & proliferation >
earliest asbestosis lesions occur in, progress to _____ respiratory bronchioles >> lung bases and slubpleura region have FIBROSIS
CXR pattern in asbestos*linear streaking*, lung bases >>> cyst fomration/honeycombining in severe
classic histo fea of berylliosisNONcaseating granuloma (mimics sarcoid)
diagnosis of berylliosis“beryllium lymphocyte transformation test” - lavage shows lymphocyte activation in exposure to beryllium salt
antigen size of hypersensitivity pneumonitis (HP)less than 3 um
pathophys of hypersensitivity pneumonitis (HP)genetic predisposition + antigen exposure >> granulomatous inflammation >> FIBROSIS (b/c M-phages unable to digest)
acute hypersensitivity pneumonitis (HP) reaction begins within ____ hours of exposure, causing ___ sx4-12 (viral like URI) —> cough/fever/malaise
PFT of acute vs chronic hypersensitivity pneumonitis (HP)acute - restriction!, chronic could show restriction/obstruction/combined
tx for hypersensitivity pneumonitis (HP)avoid exposure, corticosteroids


occupational exposures
Question Answer
ship yard and construction workers/ brake lining workers assc wAsbestos
sandblasters, rock miners, quarry workers, stonecutters assc w/silicosis
fluorescent light bulb work, aerospace, nuclear weapons, electronic assc wberylliosis
farm workers, bird breeders assc w/ hypersensitivity pneumonitis (HP)

Interstitial Lung Disease

Question Answer
2 pathologic components1) inflammation in alveolar wall/spaces “alveoli’s” 2) scarring/fibrosis
change in lung compliance / lung volumes / diffusionall decrease
*** change in gas exchangeusually hypoxemia (b/c alveolar destruction is uneven) - especially exertion — W/O co2 retention (compensate by increased minute ventilation)
change elastic recoilincrease! (lower volume at same pressure
change in RV vs TLC: TLC changes more than RV (b/c RV depends more on expiratory muscle strength)
Question Answer
change in FRCdecrease (FRC = is the volume of air present in the lungs at the end of passive expiration.)
FEV1/FVCusually normal (or increased b/c of TETHERING)
exertion hypoxemia due toDIFFUSION impairment (Fibrosis!)


Question Answer
CXR pattern of idiopathic pulmonary fibrosis (IPF)ground glass
distinguish histo of RB-ILD and DIPin DIP, see interstitial inflammation
describe organizing pneumonia (OP) histo histopathologic pattern of excessive proliferation of granulation tissue (loos collagen-embedded fiber and myofibril blasts) / bronchiolocentric/ mild chronic inflammation
*** Hamman Rich Syndrome acute interstitial pneumonia (AIP) = idiopathic DAD
Diffuse alveolar Damage assc w/ what idiopathic condition acute interstitial pneumonia (AIP)
distinguish lymphocytic interstitial pneumonia (LIP) from lymphoma*polyclonality of B cell infiltrates
sarcoid granuloma hypothesisgenetic susceptibility to exogenous agent >> granuloma
sarcoidosis more common inwomen, 20-40
sarcoid pathofphys/immmunology (primary and secondary sequelae)alveolar macrophage > recruit TH1 CD4 > pro inflammatory & profibrotic cytokines // seondayr = ANERGY
Lofgren syndromeacute sarcoidosis w/ erythema nodosum & bilateral hilar lymphadenopathy


Question Answer
Langerhans cell histiocytosis most common inyoung adults (20-40)
Langerhans cell originate frommonocyte-macrophage
pulmonary Lymphangioleiomyomatosis histosmooth muscle cells and cysts
pulmonary Lymphangioleiomyomatosis - occurs almost exclusively inwomen, childbearing age
pulmonary Lymphangioleiomyomatosis - genetic associationtuberous sclerosis complex (TSC) - mutation of TSC1 OR TSC2
CXR pattern of Chronic Eosinophilic Pneumonia: peripheral distribution pattern of infiltrates = “photographic negative o pulmonary edema”


Question Answer
tx for Chronic Eosinophilic Pneumoniacorticosteroids
usually unresponsive to anti-inflammatoryidiopathic pulmonary fibrosis (IPF)
***Pirfenidone(IPF) antifibrotic (blocks TGF-b and fibroblast proliferation) >> slows IPF progression
****Nintedanib(IPF) blocks multiple TYROSINE KINASES that mediate PDGF, VEGF, FGF,

PATHOLOGY of Interstitial Lung Disease

Question Answer
theory of fibrobrosis pathogenesisabnormal wound healing- injury > injury to TYPE I > (inflammation) and FIBROSIS
***usual interstitial pneumonia (UIP) + idiopathic origin =idiopathic pulmonary fibrosis (IPF)
usual interstitial pneumonia (UIP) - 2 key hits features1) PATCHY fibrosis - esp beneath pleura + interlobular septa 2) TEMPORAL HETEROGENEITY >> FIBROBLAST focus (loose - blue grey)
prognosis for cellular vs fibrosing nonspecific interstitial pneumonia (NSIP)cellular > fibrosing b/c responds to steroids
differentiate 2 patterns of nonspecific interstitial pneumonia (NSIP)CELLULAR (diffuse inflammatory cell infiltrate, no fibrosis) vs FIBROSING (diffuse/ temporally uniform/architecture preserved)
differentiate fibrosing NSIP from UIPdiffuse/temporal uniformity vs PATCHY/Temporal heterogeneity
common etiology of NSIPcollagen vascular disease


Question Answer
****lymphocytic interstitial pneumonia (LIP) assc w/ what 2 underlying conditionsHIV, Sjogren’s
****classic radiologic fea. of lymphocytic interstitial pneumonia (LIP)GROUND glass change (w/ cuts)
lymphocytic interstitial pneumonia (LIP) histo patternmarked LYMPHOCYTE infiltration w/ EXPANDING alveolar septa
****classic radiologic fea. of sarcoidosisBILATERAL HILAR (and MEDIASTINAL) adenopathy
****assc w/ elevated serum angiotensin converting enzymesarcoidosis
describe lymphangitic distribution and assc.interstitial location near bronchioles/vessles/pleura/septa - seen tie non-nec. granulomas of SARCOID
diagnosis of sarcoidEXLUSION (of TB, berylliosis, HYPERsensitivity pneumonitis)
assc w/ thermofilic bacteria/fungihypersensitivity pneumonitis
occupations assc w/ hypersensitivity pneumonitis (HSP)BIRD work, cheese washer lung, farmer’s lung, “magic names”
***hypersensitivity pneumonitis (HSP) triadchronic bronchiolitis / POORLY-FORMED granuloma, organizing pneumonia
natural history of hypersensitivity pneumonitis HSPcan evolve into diffuse fibrosis


Question Answer
Respiratory Bronchiolitis vs RB-ILDRB- seen with any smoker, accumulation of brown-pigment macrophages vs RB-ILD - have disease w/ RB as exclusive finding
****classic path findings of Desquamative Interstitial Pneumonia (DIP) -3(think diffuse RB-ILD) 1) idntraalveolar m-phage w/ brown pigment 2) minimal changes in septa 3) fibrosis ABSENT
classic path findings of langerhans c. histiocytosis (4)STELLATE fibrosis (bronchioles) / LANGERHANS cells, EOSINOPHILS, **BIRBECK GRANULE** - on EM
EM birbeck granule assc w/ langerhans c. histiocytosis
2 manifestations of asbestos diseasebliateral/calcified plaques & parenchymal fibrosis >> asbestosis >> honeycomb lung
2 malignancies assc w/ asbestoslung carcinoma, mesothelioma
occupations - ship yard, insulator, pipefitter assc w/Asbestosis
***where does asbestosis damage beginLOWER lungs (contrast to coal/silica exposure)
occupation - stone cutting, glass manufacturing, miner assc w/silicosis
****susceptibility to TB w/ _____ (and why?)silicosis - b/c causes damage to m-phages
damage to lung in silicosis begins whereUpper zones
classic histo fea. of silicosisstellate NODULES (esp upper zones) >>> progress to collagenous scars present in hilar nodes and pleura >> eggshell calcification on X-ray
****caplan syndrome - assc w/Coal workers’ pneumoconiosis + rheumatoid arthritis
Coal workers’ pneumoconiosis (CWP) begins whereupper lung, near respiratory bronchioles