Term
| explain the development of the lungs |
|
Definition
outgrowth of ventral foregut makes trachea and two lung bunds
right becomes 3 main bronchi and left 2 main bronchi
main bronchi (cardilage and glands) branch into bronchioles (no cartilage and glands0
bronchioles divide into terminal bronchioles, acinus, respiratory bronchioles, alveolar ducts, alveolar sacs, and alveoli |
|
|
Term
| explain the composition of the alveolar wall and septa |
|
Definition
capillary endothelium > BM > pulmonary interstitium (elastic, collagen, fibroblasts, smooth muscle, mast cells, monocytes) > alveolar epilium
alveolar epithelium has 95% type 1 pneumocytes and 5% type 2 (make surfactant, stem cell)
pores of kohn: perforations in alveolar wall for air, bacteria, exudate movement
macrophages |
|
|
Term
| cause and effect of atelectasis |
|
Definition
loss of lung volume due to inadequate expansion of air space
shunts poorly O2 blood from pulmonary arteries to veins causing V/P mismatch and hypoxia |
|
|
Term
| 3 types of atelectasis and their prognosis |
|
Definition
reabsorption: potentially reversible
compressive (passive/relaxation): not potentially reversible
contraction (cicatrization): potentially reversible
treat promptly to prevent hypoxemia and superimposed infection of collapsed lung |
|
|
Term
| reabsorption atelectasis: causes 6, effect |
|
Definition
mucoous or purulent plug in bronchus, post-op, asthma, bronchiectasis, chronic bronchitis, aspiration of foreign body
obstruction prevents air from reaching distal airways |
|
|
Term
| compression atelectasis: causes 3 |
|
Definition
accumulation of fluid, blood or air in pleural cavity causes mechanical obstruction
compression: pleural effusion caused by CHF, pneumothorax
basal: elevated diaphragm in bed ridden patient with ascites |
|
|
Term
| contraction atelectasis: cause and effet |
|
Definition
| local or general fibrotic changes hamper expansion or increase elastic recoil |
|
|
Term
| 3 signs of acute lung injury |
|
Definition
acute onset hypoxia and hypoxemia
BL pulmonary infiltrate on CXR
no clinical evidence of pirmary left sided heart failure |
|
|
Term
| 2 causes of acute lung injury |
|
Definition
non-cardiogenic pulmonary edema
acute respiratory distress syndrome - most severe |
|
|
Term
|
Definition
pneumonia
sepsis
aspritation of gastric content
severe shock or trauma
RDS of newborn |
|
|
Term
| uncommon causes of RDS 10 |
|
Definition
pulmonary contusion
fat emboli
near drowning
inhalation injury
reperfusion injury after lung transplant
cardiopulmonary bypass
acute pancreatitis
drug OD
transfusion of blood products
uremia |
|
|
Term
|
Definition
rapid onset within 72h
white out on CXR
threatning respiratory insufficiency
cyanosis
hypoxemia refractory to tx
progression to organ failure
mortality 60% |
|
|
Term
| signs of RDS in newborn 5 |
|
Definition
PDA
necrotizing entercolitis
supplemental O2: retinal injury, blindness, lung damage, bronchipulmonary dysplasia |
|
|
Term
| explain the pathology of RDS in adult |
|
Definition
imbalance of pro-inflammatory (neutrophils, iL8) and anti-inflammatory mediators causes diffuse alveolar capillary and epithelial damage
endothelial/epithelial injury compormises membrane causing protein leaking, flooding, loss of permeability, loss of surfactant
formation of hayline membranes makes alveoli sticky and increases surface tension causing collapse |
|
|
Term
| explain the pathology of RDS in the newborn |
|
Definition
| inadequate surfactant without high lectin:sphingomyelin due to immature (<34wk gestation) type II pneumocytes decreases surface tension making it difficult to inhale after each exhale and collapse of alveoi |
|
|
Term
| 3 causes of RDS of newborn |
|
Definition
prematurity (<34wk gestation) causes premature type II pneumocytes
C section: no stress so no steroid production to induce surfactant boost
maternal diabetes: increased glucose increases baby insulin which inhibits surfactant |
|
|
Term
| what does the lung look like int he acute phase of RDS 3 |
|
Definition
congestion: red, firm, airless
necrosis of alveolar epithelial cells
hayline membrane formation on alveolar ducts (edema and necrosis) |
|
|
Term
| what does the lung look like in the organizing phase of RDS 4 |
|
Definition
proliferation of type II pneumocytes to regenerate alveolar lining
intra-alveolar fibrosis
thickening of septa
deposition of collagen |
|
|
Term
| what is the prognosis of RDS 3 |
|
Definition
poor if older, bacteremia, multisystem failure
chronic sequale: difuse interstitial fibrosis, compormised function
normally function returns in 6-12mo |
|
|
Term
| what is FEV and FEC, how are they used to make diagnosis |
|
Definition
FVC: full volume exhaled timed (normal 4-6sec)
FEV: volume exhaled in 1 sec
FEV/FEC <70% means obstrivtive lung
actual FVC / perdicted FVC <80% means restricted lung |
|
|
Term
| define obstructive lung disease |
|
Definition
lung does not empty and air is trapped due to narrowing or decreased recoil
decreased: FVC, FEV, FEV:FEC ratio
increased: TLC |
|
|
Term
| COPD: moephology, cause, epidemology |
|
Definition
emphysema and chronic bronchitis
trigger: smoking
affects > 10% of adults in US
irreversible
4th leading COD in US |
|
|
Term
| what are the 4 types of emphysema |
|
Definition
centracinal / centriolobular
paracinal / pan lobular
distal acinar / paraseptal
irregular |
|
|
Term
|
Definition
|
|
Term
| what is the most common type of emphysema |
|
Definition
| centracinal 20x more common |
|
|
Term
| which types of emphysema cause significant airway obstruction |
|
Definition
|
|
Term
|
Definition
| abnormal permanent enlargement of air spaces distal to terminal bronchioles and wall destruction without significant fibrosis |
|
|
Term
| centracinal emphysema: location |
|
Definition
central/proximal alveoli (not whole lobule)
distal spread unless severe
common in upper lobes |
|
|
Term
| centracinal emphysema: cause, morphology |
|
Definition
smoking
deep pink, less lung volume |
|
|
Term
| paracinal emphysema: location |
|
Definition
uniform enlargement of all acini in a lobule from respiratory bronchiole to terminal blind alveoli
usually in ower lung zones |
|
|
Term
| paracinal emphysema: cause, morphology |
|
Definition
a1-antitrypsin deficiency
pale, voluminous lungs
obscure heart of CXR |
|
|
Term
| distal acinar emphysema: location |
|
Definition
distal acinus (esp pleura), lobular septa, margin of lobules, upper lung
adjacent to fibrosis, scaring, atelectasis
cystic sturctures that can merge and enlarge into bullae |
|
|
Term
| distal acinar emphysema cause |
|
Definition
| spontaneous pneumothorax in young people |
|
|
Term
| irregular emphysema: location, signs |
|
Definition
acinus, irregular pattern, associated with scaring
asymptomatic |
|
|
Term
| what is the underlying pathological cause of emphysema |
|
Definition
| imbalance of proteases and antiproteases, specifically antielastin |
|
|
Term
| explain how too much protease causes emphysema |
|
Definition
smokers bring toxins into lung which increases inflammation (activates neutrophils and macrophages) and generates more proteases (like antielastin from macrophages) than antiproteases leading to damage
smoke also has ROS which add to neutrophil rOS stoppig antiproteases
this causes central acinal emphysema which is more prominent in upper lobes because smoke rises in lungs |
|
|
Term
| explain how too little antiprotease causes emphysema |
|
Definition
genetic A1AT deficiency allows for every day inflammation to cause damage to alveolar air sac elastin
inflammation also release ROS from WBC which induces macrophage antielastase which damages mesenchyma and epithelium
this happens through out lung causing panacinar emphysema that is most severe in lower lobes |
|
|
Term
| why do patients with A1AT deficiency also have liver problems, how is it identified |
|
Definition
A1AT is made in liver so misfolding causes it to be stuck in hepatocye ER and cause liver damage
PAS+ globules in hepatocytes |
|
|
Term
| howis severity of A1AT deficiency determined |
|
Definition
heterozygote (PiM- normal / PiZ- mutated): decreased A1AT, risk of emphysema and liver cirrhosis if a smoker
homozygote (PiZ/PiZ): risk of panacinar emphysema and liver cirrhosis |
|
|
Term
| what are two less common genetic associations with emphysema |
|
Definition
TGF-B induces polymorphisms that could stop COPD via mesenchymal cells
MMP seen in high levels in emphysema |
|
|
Term
| how is emphysema an obstructive disease |
|
Definition
damage to air sacs causes larger spaces (rather than small alveoli) that fill with air, these spaces no longer have same ability to recoil
the recoil is what holds the small airway open (because they reocil in opposite directions). if there is no recoil there is obstruction and air trapping |
|
|
Term
| what type of morphology would you see with emphysema |
|
Definition
destoried alveolar walls leading to large air spaces
no fibrosis - wound repair cant keep up
decreased alveolar capillaries due to destruction
loss of elastin deforms terminal and respiratory bronchiole
alveolar septa collapse in expiration |
|
|
Term
| clinical signs of emphysema 13 |
|
Definition
pink puffers: expire against pursed lips to increase back pressure forcing small bronchioles open so it is easier to let air out (oxygenated still so pink) which helps with progressive dyspnea
excess work to do this causes weight loss
often old, thin, quite chext
CXR hyperinflated
flat diaphragm
sitting hunched to squeeze air out of lung
barrel chest due to prolonged exhale
secondary pulmonary HTN
hypoxia induced vascular spasm
loss of pulmonary capillary surface area
cor pulmonale |
|
|
Term
| 4 types of non-classic emphysema and their cause |
|
Definition
compensatory: dilation of alveoli in response to loss of substance elsewhere
obstructive over-inflation: lung expands because air is trapped in it (could compress functional lung)
bullous: large subpleural bullae, may rupture causing pneumothorax
mediastinal (interstitial): air enters CT stroma due to increased pressure (vomit.cough.perforation injury) causing tear and allowing air into interstitium then SC tissue causing pt to look puffy |
|
|
Term
| cause and types of chronic bronchitis |
|
Definition
simple chronic: airflow not obstructed
chronic asthmatic: hyperresponsive airway with intermittent bronchospasm and wheezing
chronic obstructive: chronic outflow obstruction associated with emphysema and smoking or air polution (SO2, NO2) |
|
|
Term
| explain the pathological changes of chronic bronchitis |
|
Definition
small airway disease
smoking brings pollutant into lung and it responds with hyperplasia and hypertrophy of mucous glands on goblet cells in trachea and bronchi, the increase makes them take up >50% airway wlal thickness (reid index normally <40%)
mucous goes into luen and is coughed up or plugs airway and traps air in the lung
this decreases oxygen delivery and traps CO2 in the lung causing cyanosis
over time inflammation infiltration (CD8, macrophages, eosinophils only in ashtma) and the toxins cause fibrosis of bronchiolar wall
coexistant emphysema causes significant airway obstruction
secondary microbe infection common and maintains inflammation and exacerbates symptoms |
|
|
Term
| explain the morphology of chronic bronchitis |
|
Definition
lining hyperemic, swollen
increased secretions and enlargement of mucous glands
small airway disease: goblet metaplasia, mucous plugging, fibrosis, inflammation
severe: complete lumen oblideration (bronchiolitis obliterans) |
|
|
Term
| clinical signs of chronic bronchitis 15 |
|
Definition
persistent productive cough for 3 consecutive months in 2 consecutive years
cough: due to excess mucous production
blue bloater: mucous plug traps CO2 and decreases O2, overweight, cyanotic, elevated Hb, peripheral edema, ronchi, wheezing
chronic outflow obstruction: hypercapnia, hypoxemia, cyanosis
secondary pulmonary HTN, right sided heart failure, CHF cor pulmonale
recurrent infection |
|
|
Term
| what is the most important reason for pulmonary induced pulmonary HTN |
|
Definition
| arterioles clamp due to lack of O2 to shunt blood in a disease that is wide spread so too many areas clamp |
|
|
Term
| what are the 4 stages to asthma |
|
Definition
1. genetic predisposition to type I hypersensitivity
2. allergen introduction activates CD4 Th2
3. allergen re-introduction: early and late phase reactions
4. long term: atelectasis and airway remodeling |
|
|
Term
| what are the 6 genetic mutations associated with asthma |
|
Definition
CH5q: upregulate IgE synthesis, mast cell, and eosinophil growth
ILI3: susceptability to atopic asthma
CD14: mutation associated with occupational asthma
Class II HLA mutation: makes IgE antibodies
IL-4 receptor: increases IgE causing atopic asthma
CH20q ADAM-33: regulator of smooth muscle and fibroblast remodeling |
|
|
Term
| on initial introduction of allergen what does Th2 CD4 release (3), what do they do |
|
Definition
IL4: induce CH5q to cause class switch to IgE
IL-5: attract eosinophils
IL-10: stimulate TH2 and inhibit TH1 synthesis |
|
|
Term
| what happens in the early phase reaction |
|
Definition
IgE stimulate degranulation of mast cells which release histamine which causes vasodilation and increases permeability. loss of protein leads to edema
histamine induces leukotrienes (CD4, D4, E4) which cause vasovonstriction, permeability, and edema, inflammation, bronchoconstriction |
|
|
Term
| what happens in the late phase reaction |
|
Definition
eosinophils release major basic protein which damages cells and increases bronchoconstriction
mucous plugs occlude bronchi, formation of curshmann spirals and charcot leyden crystals
overall amplification of inflammatory response via eosinophils, neutrophils, TH2, chemockines (recruit more TH2) |
|
|
Term
| what are curshmann spirals |
|
Definition
| whorls of shed epithelium in mucous plugs seen in asthma |
|
|
Term
| what are charcot leyden crystals |
|
Definition
| crystalloids with eosinophil proteins in mucous plug seen in asthma |
|
|
Term
| what are the 6 areas of remodeling that occur in asthma |
|
Definition
hypertrophy and hyperplasia of bronchial smooth muscle
deposition of subepithelial collagen and angiogenesis in submucosa
subbasement membrane fibrosis
increased size of submucosal glands
goblet metaplasia |
|
|
Term
| what are the symptoms of asthma |
|
Definition
episodes: wheezing, breathlessness, tight chest, cough (esp night), difficult expiration
attack: severe dyspnea and wheeze
status asthmaticus: severe attack that won't respond to therapy
late phase reaction: amplification of inflammatory response 4-8h later
hygiene hypothesis: infection alters immune homeostasis and promotes allergic response |
|
|
Term
| asthma attack: define, triggers, pathology, length, tx |
|
Definition
dyspnea and wheeze due to bronchospasm
triggers: smoke, fumes, cold air, exercise, infection
1 - several hours
TX: bronchodilators, corticosteroids |
|
|
Term
| status asthmaticus: define, length, 3 signs |
|
Definition
severe asthma attack that wont respond to therapy and may be fata
days - weeks long
hyper capnia, acidosis, severe hypoxemia |
|
|
Term
| what are the 4 different kinds of asthma |
|
Definition
extrinsic/atopic
intrinsic/non-atopic
drung induced
occupational |
|
|
Term
| triggers of atopic asthma |
|
Definition
| pollen, dust, dander, foods |
|
|
Term
| pathological effects of atopic asthma |
|
Definition
| excess IgE/TH2 response to environmental antigen, often familial, needs prior sensitization |
|
|
Term
| how is atopic asthma diagnosed |
|
Definition
| wheel and flare reaction immediatly on skin test |
|
|
Term
|
Definition
begins in childhood
warniing signs; allergic rhinitis, uticaria, eczema
sensitization causes immediate response upon re-exposure of bronchoconstriction, edema, mucous |
|
|
Term
| trigger for intrinsic asthma |
|
Definition
non immune: ASA, cold, exercise, psych, pollution (SO2, NO2, Ozone)
virus: rhino, PIV decrease subepithelial vagus receptor |
|
|
Term
| diagnosis of intrinsic asthma |
|
Definition
| skin test usually negative |
|
|
Term
|
Definition
| common end pathway as atopic asthma so treated the same |
|
|
Term
| what is the most common cause of drug induced asthma, what is the physiological response |
|
Definition
| aspirin inhibits COX shifting balance toward lipooxygenase causing bronchial spasm |
|
|
Term
| triggers of occupational asthma |
|
Definition
fumes: eopxy resins, plastics
chemical dust: wood, cotton, platinum
gas: tolurene |
|
|
Term
| signs of occupational asthma |
|
Definition
usually develops after repeated exposure
goes away on weekend |
|
|
Term
| 6 causes of bronchiectasis |
|
Definition
bronchial obstruction (local): tumor, foreign body mucous
cystic fibrosis (diffuse): obstruction via viscous mucous secretion and infection risk
immune deficiency (local or diffuse): increased susceptability to infection
kartagner syndrome (AR): abnormal cilia impair clearance increasing infection and decreasing sperm motility
necrotizing pneumonia: S. aureus, klebsiella, post-TB
allergic bronchopulmonary aspergillosis: hypersensitivity to aspergullus causes chrinic inflammatory damage, usually in people wit asthma and CF |
|
|
Term
| what pathological changes occur in brochiectasis |
|
Definition
obstruction and chronic infection
damages bronchial walls causing weakness, dilation, cilia destruction
decreased microbe clearance increases infection
acute and chronic inflammatory exudate in walls of bronchi and bronchioles causes desqumation and ulceration with mixed flora
permanent dilation of bronchi and bronchioles due to destruction of muscle and elastic supporting tissue
permant dilation and scaring, fibrosis of walls, peribronchiolar fibrosis, necrosis of bronchiolar walls leads to abscess and fungi development |
|
|
Term
| locations of bronchiectasis |
|
Definition
| lower lobes (more vertical), bilateral |
|
|
Term
| CXR signs of bronchiectasis |
|
Definition
| airway dilation almost to pleural surfaces |
|
|
Term
| clinical signs of bronchiectasis |
|
Definition
dilated airway causes mucous to sit causing mucopurulent cough, foul smelling sputum, CO2 build up and hypoxemia
hypoxemia causes shunting and pulmonary HTN leading to cor pulmonale, finger clubbing
secondary amyloidosis: chronic inflammation causes over production of SAA which makes AA which deposits amyloid in the lung (deposition of parts of IgG from C cells)
in infectious: metastastic brain abscess, uRI, risk of more infection |
|
|
Term
| chronic bronchitis: anatomical site, major pathology changes 2, causes 2, signs 2 |
|
Definition
bronchus
mucous hyperplasia and hypersecretion
tobacco, air pollution
cough, sputum |
|
|
Term
| bronchiectasis: anatomical site, major pathology change s2, cause, 3 signs |
|
Definition
brnchys
airway dilation, scaring
persistant infection
cough, purulent sputum, fever |
|
|
Term
| asthma: anatomical site, pathological changes 3, cause 2, 3 signs |
|
Definition
bronchys
smooth muscle hyperplasia, mucous, inflammation
immunologic or undefined
wheeze, cough, dyspnea, episodic |
|
|
Term
| emphysema: anatomical site, major pathological changes 2, cause, sign |
|
Definition
acinus
airspace enlargement, wall destruction
tobacco smoke
dyspnea |
|
|
Term
| Restrictive lung disease: definition, changes in lung volumes |
|
Definition
restricted filling and expansion of parenchyma
reduced lung compliance (stiff) causes dyspnea and chage in V/P ratio hypoxia
decreased: TLC, FEV, FVC
increased FEV:FVC (because they both decrease but FVC decreases more |
|
|
Term
| causes of restrictive lung disease |
|
Definition
chest wall abnormality: obesity, pleural disease, NMJ disorder
interstitial/intraalveolar build up of interstitium components due to disease
acute interstitial lung disease: ARDS
chronic interstitial lung disease: pneumoconiosis, idiopathic pulmonary fibrosis, infiltrative (sarcoidosis) |
|
|
Term
| clnical signs of restrictive/infiltrative lung disease |
|
Definition
dyspnea: increased breathing effort and hypoxia
complications: cor pulmonale, pulmonary HTN, scaring (honeycomb lung9 |
|
|
Term
| CXR appearance of restrictive/infiltrative lung disease |
|
Definition
diffuse small nodules
irregular lines
ground glass shadows |
|
|
Term
| in comparison to obstructive diseases when do restrictive diseases appear |
|
Definition
|
|
Term
| pathogenesis of restrictive diseases |
|
Definition
persistant inflammation: lymphocytes, macrophages, neutrophils, case parenchymal injury, fibroblast proliferation, fibrosis
activation of pulmonary macrophages: key in pathogenesis of fibrosis |
|
|
Term
| what are the fibroing diseases |
|
Definition
| usual interstitial pneumonia / idiopathic pulmonary fibrosis |
|
|
Term
|
Definition
|
|
Term
| explain the pathogenesis of UIP |
|
Definition
TGF-B1 mutation causes increased release which injures pneumocytes causing chronic epithelial injury
TPF mutation shortens telomerases causing apoptosis
chronic injury and apoptosis activates TH2, eosinophils, and mast cells which go to ALVEOLAR SEPTA
injured type II pneumocytes then release more mutated TGF-B1 which cause abnormal repair |
|
|
Term
| explain the progression of the morphology of abnormal repair in UIP |
|
Definition
fibroblastic foci (early lesion): TGF dwn regulates caveolin-1 (inhibitor of pulmonary ibrosis) allowing myelofibroblast proliferation leadiing to fibrosis of septa (firm,rubery) that varies in intensity over time
temporal heterogeneity; fibroblastfoci becomes collagenous
honeycomb: patchy interstitial fibrosis collapses alveolar walls and forms cystic spaces with hyperplastic type II pneumocytes
cobblestone pleura: due to retraction of SCARS ON INTERLOBULAR SEPTA |
|
|
Term
|
Definition
nonproductive cough
progressive dyspnea
cyanosis
cor pulmonale
edema later
gradual onset |
|
|
Term
|
Definition
relentless progression
3y survival
lung transplant needed |
|
|
Term
| nonspecific interstitial pneumonia pathogenesis |
|
Definition
diffuse fibrosing interstitial lung disease
cellular or fibrosing pattern (patchy fibrosis without temporal heterogenicity)
fibroblastic honeycombing |
|
|
Term
| signs of nonspecific interstitial pneumonia |
|
Definition
dyspnea and cough for several months
chronic, bilateral, interstitial |
|
|
Term
| prognosis of non specific interstitial pneumonia |
|
Definition
| cellular pattern is bettwe than fibrosing but fibrosing is still better than UIP |
|
|
Term
| cytogenic organizing pneumonia aka |
|
Definition
| BOOP: broncholitis obliterans organizing pneumonia |
|
|
Term
| cytogenic organizing pneumonia pathogenesis |
|
Definition
response to infection or inflammatory injury (vascular disease, transplant)
polyploidy plugs of loose organizing CT in alveoli, alveolar ducts, bronchioles
alveolar spaces fill with fibroblasts
CT is same age and underlying architecture is normal |
|
|
Term
| symptoms of cytogenic organizing pneumonia |
|
Definition
|
|
Term
| CXR cytogenic organizing pneumonia |
|
Definition
| ubpleural or peribronchial patchy areas of airspace consolidation |
|
|
Term
| TX cytogenic organizing pneumonia |
|
Definition
| steroid tx for 6mo or longer (RO organizing pneumonia causes) |
|
|
Term
| what are the patterns of association of vascular disease with fibrosing lung disease |
|
Definition
| UIP, NDIP, vascular sclerosis, organizing pneumonia, bronchiolitis, pleural |
|
|
Term
| what are the causes of the association of collagen vascular disease with fibrosing lung disease |
|
Definition
| RA, SLE, systemic sclerosis, dermatomyomyositis-polumoysitis |
|
|
Term
| prognosis of collagen vascular disease + fibrosing lung disease |
|
Definition
| poor prognosis, but better than UIP |
|
|
Term
| pathogenesis of pneumoconiosis |
|
Definition
non-neoplastic reaction to inhalation of mineral dust, organic particles, fume, vapor (worsened by smoking)
macrophages endocytose trapped particles and cause fibrosis to wall them off because they are unable to digest them
lymph drainage and macrophage migration amplifies local reaction |
|
|
Term
| what are the differences between the causes of pneumoconiosis that affect the severity |
|
Definition
smoking worsens effects of mineral dusts (esp asbestos)
size (1-5micron most dangerous and lodge in distal airway), shape, solubility, and particle reactivity
coal dust is larger and disease has later onset, silica, abestos, beryllum are smaller and cause fibrosis at lower concentrations |
|
|
Term
| berylliosis: cause, morphology 3, Prognosis |
|
Definition
beryllium miners, aerospace workers
noncaseating granuloma of lung, hilar nodes, organs (sarcoidosis like)
increased risk for lung cancer |
|
|
Term
| what are the 4 types of coal worker pneumobicosis |
|
Definition
asymptomatic anthrocosis
simple coal worker pneumonociosis
complicated CWP
progressive massive fibrosis |
|
|
Term
| asymptomatic anthrocosis: morphology, cause |
|
Definition
| seen in urban dwellers and smokers, pigment engulfed by macrophages will deposit in CT, lymphatics, and nodes without cellular reaction |
|
|
Term
| simple coal workers pneumonicosis: cause, morphology, symptoms |
|
Definition
accumulation of macrophages in coal macules (dust laden macrophages) and coal nodules (collagen fibers) in UPPER lung zone
little pulmonary dysfunction, possible centrolobular emphysema |
|
|
Term
| complicated CWP or progressive massive fibrosis pathogenesis |
|
Definition
| coalescence of coal nodules over years cause smultiple blackened scars (fibrosis) 2-10cm with dense collagen and pigment |
|
|
Term
| complicated CWP or progressive massive fibrosis prognosis |
|
Definition
<10% of simple CWP will progress to PMF
progression can occur without further exposure, with increased exposure, increased total dust burden, quality of exposure, smoking, genetics |
|
|
Term
|
Definition
increasing pulmonary dysfunction
pulmonary HTN
cor pulmonale
caplan syndrome: RA and pneumoconosos |
|
|
Term
| what is the prognosis of CWP |
|
Definition
usually benign
if PMF: increased pulmonary dysfunction, pulmonary HTN, cor pulmonale
NO INCREASED RISK OF BRONCHOGENIC CARCINOMA
arthracosis: minor carbon pollution has little clinical effect |
|
|
Term
|
Definition
| crystalline silica (worse than amorphus) often quarts activate rekease of mediators by pulmonary macrophages causing fibrosis |
|
|
Term
|
Definition
silicotic nodules: small, pale black, concentric hyalinized collage around amorphous center creates whorled appearance in UPPER lung zones
nodules coalesce into hard collagenous scar which compresses parenchyma and causes honeycomb
fibrotic lesions occur in HILAR nodes (eggshell calcification around uncalcified region of caseation) |
|
|
Term
| how are silica fibers identified |
|
Definition
| microscopically bifringent and polarized |
|
|
Term
|
Definition
| fine nodularity of upper lungs |
|
|
Term
|
Definition
asymptomatic until PMF
have progressive dyspnea, pulmonary HTN, cor pulmonale |
|
|
Term
|
Definition
increased risk of TB (silica inhibits phagolysosome formation)
may progress of PMF
crystalline silica is carcinogenic |
|
|
Term
| what are the two forms of asbestos |
|
Definition
| crystalline hydrataed silicate with fibrous geometry in serpentine (chrystile) or amphibole form |
|
|
Term
| serpentine/chrysolite: shape, properities, movement in body |
|
Definition
curly, flexible
more soluble if gets down into lung leaks into tissues
structure allows it to get stuck in upper respiratory passage and removed by mucocilliary elevator usually |
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Term
| amphiboles: shape, properities, movement in body |
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Definition
less common but more pathogenic
stiff, brittle, less soluble
structure causes it to get further into lung past ciliary elevator and stick in epithelium and interstitium (not soluble) increasing risk for lung cancer and mesothelioma |
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Term
|
Definition
| release of ROS and toxins induces production of tumors (bronchogenic carcinoma, laryngeal carcinoma, malignant pleural and peritoneal mesothelioma) |
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Term
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Definition
interact with particles in macrophages causing interstitial fibrosis and pleural fibrosis/plaque on parietal pleura (no asbestos bodies) which can contract casing honeycombs
asbestos bodies: golden brown filsuform or beaded rods, translucent center, coated with Fe proteinaceous mineral formed when macrophages to kill
begins in LOWER lobes and SUBPLEURALLY and moves upward |
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Term
| clinical signs of asbestos |
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Definition
progressive dyspnea 10-20y after exposure
PRODUCTIVE cough
static or progressive to CHF, cor plmonale, death
bronchiogenic carcinoma (x55 risk if smoker, more common)
mesotheliona (1000x risk, no associated with smoking, less common |
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Term
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Definition
| pleural plaques cause circumscribed densities |
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Term
| what does bleomycin (chemo) and amiodarone do to the lungs, why |
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Definition
| pneumonitis, fibrosis due to recruitment of inflammatory cells to alveoli |
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Term
| what does acute radiation do to lungs, signs, tx |
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Definition
1-6mo after therapy (usually thorax tumor)
fever, dyspnea (out of proortion), pleural effisions, pulmonaly infiltrates
resolves with steroids |
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Term
| chronic radiation pneumonitis: cause, morphology |
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Definition
progression of acute radiation pneumonitis
associated with pulmonary fibrosis |
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Term
| what are the 2 granulomatous diseases |
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Definition
sarcoidosis
hypersensitivity pneumonitis / allergic alveolitis |
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Term
| epidemology sarcoidosis 5 |
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Definition
<40yo
high in danish, swedish, AA, non smokers |
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Term
| pathogenesis of sarcoidosis |
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Definition
"disordered immune regulation" genetic oligoclonal expansion of TH1 cell receptor striggered by enviornmental causes lead to TH1 cell accumulation in intraalveolar space and release of cytokines (IL2, INFy, macrophage inflammatory protein 1a)
activates macrophages (recruits more T cells, creates ultisystem noncaseating granulomas)
polyclonal hypergammaglobulineaia
bilateral hilar lymphadenopathy |
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Term
| what are the possible environmental triggers of sarcoidosis 5 |
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Definition
| viruses, mycobacteria, borrella, pollen but there is no evidence suggesting infectious cause |
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Term
| what areas of the body does sarcoidosis effect |
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Definition
lymphangitic dystribution:
lung
hilar nodes
parotid glands
skin
eye
lacrimal glands
spleen
liver
marrow |
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Term
| sarcoidosis: morphology in lung |
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Definition
noncaseating granuloma derived from macrophages with CD4 rim and eosinophilic
laminated fibrosis > diffuse interstitial fibrosis > collagen > hayalnized scar > honeocomb lung
schumman bodies: laminated concentration of Ca and protein
necrotic foci: occurs when granuloma acquires secondary infection
multinucleated giant cells formed by macrophage fusion
asteroid bodies: stellate inclusion in giant cells of granulomas |
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Term
| effect of sarcoidosis on lymph nodes and glands |
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Definition
BL hilar lymphadenomathy, paratracheal: painless, firm, rubbery, nonmatter, no ulceration
unilateral or BL enlargement of parotid
decreased salivation and lacrimation |
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Term
| what is mikulicz syndrome |
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Definition
parotid enlargement and xeroxtoma
seen in sarcoidosis |
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Term
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Definition
inflammation of the lacrimal gland decreases lacrimation
seen in sarcoidosis |
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Term
| what are the 2 effects of sarcoidosis on the skin |
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Definition
erythema nodosum: raised red tender nodule on anterior leg without sarcoidal granulomas in them
SC nodules: discrete painless abundant in onocaseating granulomas |
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Term
| signs of sarcoidosis in the ey |
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Definition
iritis/iridocylitis: uni/bl corneal opacification, glaucoma, vision loss
choriditis, retinitis, optic nerve damage, uveitis |
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Term
| sigsn fo sarcoidosis in the liver, marrow, and spleen |
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Definition
spleen: granuloma, HSM
liver: microscopic granuloma in portal triad, abnormal function. HSM
marrow: granuloma asymptomatic |
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Term
| why do people with sarcoidosis get hypercalcemia and hypercalcuria |
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Definition
increased Ca absorption due to over production of vit D by mononculear phagocytes in granulomas
1a-hydroxylase activity of epitheloid histocytes converts VitD to active form (seen in all noncaseating granulomas) |
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Term
| clinical signs of sarcoidosis 16 |
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Definition
asymptomatic, usually discovered on CXR
peripherial lymphadenopathy
sternal discomfort
cutaneous lesions
eye involvement
HSM
gradual SOB, dry cough, increase ACE
hypercalcemia
hypercalcuria
fever
fatigue
weight loss
anorexia
night sweats |
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Term
|
Definition
| bilateral hilar lymphadenopathy |
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Term
|
Definition
anergy to skin tests for candidia or PPD due to pulmonary recruitment of CD4 T cells and peripheral depletion
bronchoalveolar lavage abudent in CD5 |
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Term
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Definition
reoccurs in 75% after lung transplant
can be progressive and chronic or have periods of remission
steroid therapy: can help bring on a remission, 60% recover without damage
10% have progressive pulmonary fibrosis and cor pulmonale |
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Term
| give 9 types of hypersensitivity pneumonitis and the organism associated with each |
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Definition
farmer's lung: moldy hay with M. faeni
bagassosis: sugar cane with T. actinomycetes
maple bark: Cruyptostroma corticale
humidifier lung: cool mist with T. actinomycetes
malt worker: moldy barley with aspergillus
cheese washer: moldy cheese with panicillium
pigeon breeders: pigeon with serum proteins in droppings
chemical workers: trimellite anhydride, iscocyanates |
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Term
| explain pathogenesis of allergic alveolitis |
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Definition
| inhaled antigen causes bronchial asthma. immune reaction occurs in bronchi but damages alveoli via type III (immune complex: Ab, Ig, complement) and type IV (noncaseating granuloma delayed type) hypersensitivity |
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Term
| morphology of hypersensitivity pneumonitis |
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Definition
patchy mononuclear infiltrate in INTERSTITIUM
PERIBRONCHIAL accentuation
INTERSTITIAL noncaseating granuloma PERIBRONCHULAR
diffuse INTERSTITIAL fibrosis |
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Term
| diagnosis of allergic alveolitis |
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Definition
bronchoalveolar lavage: increased CD4/CD8
serum: percipitating antibodies, complement and Ig OR OR OR non caseating granulomas |
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Term
| signs of allergic alveolitis acute reaction 4 |
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Definition
fever, cough, dyspnea
4-8H AFTER EXPOSURE |
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Term
| signs of chronic hypersensitivity pneumonitis 5 |
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Definition
slow onset
cough, dyspnea, malaise, weight loss |
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Term
| prognosis of allergic alveolitis |
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Definition
if antigen removed symptoms will clear within days
chronic exposure causes irreversible chronic interstitial pulmonay disease (fibrosis)
acute exacerbation on re-exposure |
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Term
| what is the general pathogenesis of pulmonayr eosinophilia |
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Definition
| infiltration and activation of eosinophils later elevated by alveolar IL-5 |
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Term
| what are 5 types of pulmonary eosinophilia |
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Definition
acute eosinophilic pneumonia with respiratory falure
simple pulmonary eosinophilia (loeffler syndrome)
tropical eosinophilia
secondary eosinophilia
idiopathic chronic eosinophilic pneumonia |
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Term
| acute eosinophilic pneumonia with respiratory failure: signs 5, diagnosis 1, TX 1 |
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Definition
rapid onset, fever, dyspnea, hypoxia, pylmonaly infiltrate
bronchoalveolar lavage >25% eosinophils
TX corticosteroids |
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Term
| simple pulmonary eosinophilia (loeffler sydrome) signs 3, morphology 3 |
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Definition
transient pulmonayr lesions, eosinophilia, benign course
thickened alveolar septa via eosinophil and giant cell infiltrate |
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Term
| tropical eosinophilia cause 2 |
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Definition
microfilariae
helminthic parasites |
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Term
| secondary eosinophilis is associated with 3 |
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Definition
| asthma, drug allergies, vasculitis |
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Term
| idiopathic chronic eosinophilic pneumonia: moephology 2, location 3, signs 3, diagnosis 1 |
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Definition
aggregates of lymphocytes and eosinophils
in septa and alveolar space in periphery of lung
fever, night sweats, dysnpea
disease of exclusion |
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Term
| what are two smoking related itnerstitial diseases |
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Definition
desquamative interstitial pneumonia
respiratory bronchiolitis |
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Term
| morphology desqumative interstitial pneumonia |
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Definition
alveolar septa thickened by inflammatory infiltrate (usually leukocutes) and fibrosis
smoker's macrophages: in alveolar spaces, accumulation of macrophages with abuntant cytoplasm and dusty brown pigment |
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Term
| prognosis of smoking related interstitial diseases 2 |
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Definition
desquamative interstitial pneumonia: good with smoking cessation and steroids
respiratory bronchiolitis: good with smoking cessation |
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Term
| morphology of respiratory bronchiolitis |
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Definition
pigmented intraluminal macrophages in bronchiolocentric distribution (1st and 2nd order bronchioles)
peribronchiolar fibrosis |
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Term
| signs of respiratory bronchiolitis 3 |
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Definition
gradual onset
dyspnea
dru cough |
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