Lung Path Flashcards
which bronchi is straight
right
what size particles are most dangerous
.5-5um
lobule
cluster of terminal bronchioles w/attached acini
acinus
respiratory bronchiole and all attached alveolar ducts and sacs
pulmonary hypoplasia
common 10% neonatal autopsy
seen w/fetal compression and other anomalies
congenital foregut cysts
detached section of maldeveloped foregut presents as mass or incidental finding, possibly in adulthood mediastinal and hilar locations not connected to airway usually bronchogenic w/respiratory epi some esophageal, some enteric
cystic adenomatoid malformation
CPAM
Hamartomatous lesions w/abnormal bronchiolar tissue
types of CPAM
type I- large cysts good prognosis
type II- medium cysts, poorer prognosis since associated w/other congenital malformations
bronchopulmonary sequestrations
areas of lungs w/o normal connections to airways
blood supply is from systemic aa
extralobar bronchopulmonary sequesterations
external to lung
may have other congenital anomalies
intralobar bronchopulmonary sequestrations
w/in lung
associated w/recurrent local infection and/or bronchiectasis
most likely an acquired lesion
respiratory distress in newborn
excessive maternal sedations fetal head injury blood or amniomtic fluid aspiration intraunterine hypoxia from cord hyaline membrane disese
hyaline membrane diease stats
most common
1000 deaths in 2002, but 25000/year in 60s
hyaline membrane disease
can occur in term infants, but usually preterm
rate inversely proportional to gestational age
associated w/males, materal DM, multiple gestations, and c-section before onset of labor
immature lungs
deficiency of surfactant
alveoli development
20wks- glandular
30wks- saccular
term- alveolar
bronchopulmonary dysplasia
> 28 days of O2 therapy in infant >36weeks post mentrual age
alveolar hypoplasia and thickened walls
dysmorphic capillaries and decreasesed VEGF
cytokines (TNF, IL8, etc)
increased and may have role
cystic fibrosis
widespread disorder in epi transport affecting fluid secretion in exocrine glands and epi of resp, GI, and repro tracts
viscid secretions
autosomal recessive
CFTR gene chrom 7
most common lethal genetic condition in Caucasians
Dx criteria for CF
one or more characteristic phenotypic features or a Hx or CF in sibling or positive newborn screening test
AND
an increased sweat chloride on 2+occasions or 2CFTR mutations or demonstration of abnomral epi nasal ion transport
Tx of CF- pancreas
oral pancrelipase
may progress to DM
Tx of CF- vit deficiency
oral fat soluble vitamins
parenteral nutrition
Tx of CF- pulomonary
postural drainage and chest percussion bronchcodilators mucolytic agents Antibiotics hypertonic saline high dose ibuprofen slows lung disease progression
apparent life threatening event (ALTE)
if survive at risk for future respiratory death
prolonged apnea, diminished responses to hypercarbia or hypoxia
often premature or have mechanical disorders leading to compromise
no longer considered true SIDs
atelectatsis
incomplete expansion- neonatal
acquired collapse- adults can be due to: resorption/obstruction, compression, or contraction
ALL AT RISK FOR INFECTION
resorption/obstruction atelectasis
airway obstruction, mucus, foreign body, tumor
mediastinal shift toward involved lung
compression atelectasis
external pressure including elecated diaphragm
mediastinal shift away from involved lung
contraction atelectasis
secondary to fibrosis
irreversible
types of pulmonary edema
hemodynamic
microvascular
hemodynamic pulmonary edema
most common
increased hydrostatic pressure due to left sided heart failure
basal lower lobes
heart failure cells
secondary infections
if chronic leads to alveolar fibrosis (brown lung)
microvascular (alveolar) injury pulmonary edema
increased permeability
caused by infection, toxic injry,
if diffuse leads to ARDS
non-cardiogenic pulmonary edema criteria
acute onset of dyspnea
hypoxemia
b/l infiltrates
absence of primary left heart failure
non-cardiogenic pulmonary edema etiology
wide spread disorers injuries may be related to mediators such as cytokine, oxidants, TNF, ILs, TGF beta, susceptibility may be heritable may progress to ARDS OR AIP
Adult ARDS
shock rapid onset, life threatening occurs in patients w/severe disease diffuse damage to alveolar capillary walls -> neutrophilic migration secondary loss of surfactant prothrombotic milieu
infectious conditions associated w/adult ARDS
Sepsis
diffuse pulmonary infections
gastric aspiration
physical injury ARDS
mechanical trauma, including head injuries pulmonary contusions near-drowning fractures w/fat emboli burns ionizing radiation
inhaled irritants ARDS
O2 toxicity
smoke
irritant gases and chemicals (paraquat)
other causes of ARDS
cardiac reperfusion injury
ARDS clinical
patients are already ill when ARDS superimposed rapid onset profound dyspnea and tachypnea cyanosis and respiratory failure diffuse b/l inflitrates up to 60% fatal permanent damage
acute interstitial pneumonia (AIP)
symptoms similar to ARDS no associative causative disorder 59 yrs, M=F acute respiratory failure following illness 33-74% mortality rate w/in 2 months
obstructive lung disease
limit rate of flow
FEV1/FVC reduces
due to resistance at any level
obstructive lung disease examples
COPD, chronic bronchitis, asthma
restrictive lung disease
limit total lung capacity and residual volume
near normal flow rates
restrictive lung disease examples
chest wall disorders, obesity, ARDS, interstitial fibrosis, penumoconioses
COPD classifications
centriacinar/cantrilobular
panacinar/panlobular
distal acinar/paraseptal
irregular/paracicatrical
centriacinar/centrilobular
smoking, smoking, smoking
predominately upper lobes/apices
panacinar/panlobular
alpha-1 antitrpsin deficiency, smoking
predominately ant/lower lobes
distal acinat/paraseptal
- subpleural and adjacent to septate
- may be bullous and cause spontaneous penumothrorax in young adults
- associated w/previous damaged lung
irregular/paracicatrical
common, but focal and usually asymptomatic associated w/scarring
proteolytic digrestion of alveolar walls in COPD
via neutrophil-secreted elastase
normally inhibited by alpha 1 antitrypsin
COPD clinical
symptoms after 1/3 of lung damaged barrel chest low FEV1, high TLC, and RV pink puffer bullous emphysema repsiratory acidosis cor pulmonale
COPD Tx
quit smoking bronchodilators steroids bullectomy transplant
other conditions w/increased air
compensatory hyperinflation
obstructive overinflation
interstitial emphysema
compensatory hyperinflation
due to loss of adjacent tissue
obstructive overinflation
trapped air
-ball valve obstruction by object
collaterals feeding around obstruction, life threatenting,
alveolar pores of kohn, bronchiloloalveolar canals of lambert
-congenital lobar overinflation from lack of bronchial cartilage
interstitial emphysema
any air in inetersitium
chronic bronchitis
clinically defined as 3 mo productive cough/year for 2 consecutive years smoking, smoking, smoking hypersecretion of mucus increased reid index brochiolitis obliterans in small airways superimposed infection dyspnea and cor pulmonale blue bloaters
reid index
normal <.4
thickness of gland/thickness of walls
hypertrophy of bronchial submucosal glands
age bronchitis vs COPD
40-45 VS 50-75
dyspnea bronchitis vs COPD
mild, late vs severe early
cough/sputum bronchitis vs COPD
early copious vs late scant
infections bronchitis vs COPD
common vs occasional
resp insufficiency bronchitis vs COPD
repeated vs terminal
cor pulmonale bronchitis vs COPD
common vs rare terminal
airway resistance bronchitis vs COPD
increased vs normal slightly increased
elastic recoil bronchitis vs COPD
normal vs low
chest radiograph bronchitis vs COPD
prominant vessels, large heart vs hyperinflation, small heart
appearance bronchitis vs COPD
blue bloater vs pink puffer
asthma
chronic inflammatory disorder of airwyas
reactive airspace disease w/episodic partially reversible bronchoconstriction
type I hypersensitivity asthma
atopy
begins in childhood
mucosal mast cells react
stimulation of sub epi vagal receptors
non-atopic asthma
associated w/pulmonary infections and air pollutants
no allergic indicators
infection lowers threshold for vagal response
drug induced asthma
uncommon form of disease
can also cause urticaria
aspirin classic cause ( inhibit cox 2, but not cox 1 so increases leukotrienes
other types of asthma
exercise induced
occupational
asthma morphology
epi injury subbasement membrane fibrosis eos and inflam infiltrates hypertophy/hyperplasia of submucosal glands and goblet cell metaplasia hypertrophy/hyperplasia of smooth muscle increased vascularity
Histo markers of asthma
charcot-leyden crystals
curschmann spiral
bronchiectasis
permanent dilation of bronchi and bronchioles
causes of brochiectasis
tissue distruction secondary to infection
symptoms of bronchiectasis
foul smelling +/- bloody sputum
dyspnea, orthopnea, rarely severe hemoptosis
may develop cor pulmonale, brain abscesses and amyloidosis
brochiectasis associated w/
genetic disorder obstruction many infections chronic inflammatory conditions pulmonary sequestration allergic bronchopulmonary aspergillosis autoimmune disorder and post transplant rejection of graft vs host ds
genetic disorders and brochiesctasis
CF
primary ciliary dyskinesia- kartagener syndrome: male infertility, sinusitis, situs inversus,
chronic diffuse interstitial lung diseases
restrictive
heterogenous group of disease
inflitrative x-ray changes (reticulonodular or ground glass)
most involve interstiutium and alveolar walls
symptoms of chronic diffuse interstitial disease
decreased TLC and RV dyspnea tachypnea cyanosis end-inspiratory crackles end-stage- honey comb lung
complications of restrictive disease
Cor pulmonale
pulmonary HTN
fibrosing restrictive diseases
unusual interstitial pneumonia non-sepcific interstitial pneumonia cyptogenic organizing pneumonia CT disease drug rxns radation
granulomatous restrictive diseases
sarcoidosis
hypersensativity pneumonitis
Eosinophillic
smoking related restrictive disease
- desquamative interstitial pneumonia
- respiratory broncholitis associated interstitial lung disease
other causes of restrictive disease
pulmonary langerhands cell histocytosis
pulmonary alveolar proteinosis
lymphoid interstitial pneumonia
idopathic pulmonary fibrosis (IPF) aka
unusual interstitial pneumonia (UIP)
cryptogenic fibrosing alveolitis
IPF
unknown pathogenesis
repeated injury to alveolar wall
type I pneumocyte death, type II hyperplasia
inflammation via TH2, fibroblast proliferation, TBFbeta1 collagen deposisition
environmental factors IPF
smoking
work related exposures
reflux esophagitis
clinical IPF
insidious unpredictable disease in middle age >50
dyspnea, dry cough, hypoxemia w/cyanosis, digital clubbing
mean survival <3yrs
pathology IPF
repeated cycles of alveolitis
healing/scarring -> patchy interstitial fibrosis
predominently subpleural/interlobular and lower lobe
end state: honey comb
Tx IPF
lung transplant
Non-specific interstitial penumonia (NSIP)
patients do not get as sick or progressive as IPF
better prognosis then IPF
symptoms of NSIP
dyspnea and cough for months
middle ages w/milder sympotms
non-smoking females
NSIP histo
all lesions at same stage
cryptogenic organizing pneumonia (COP) aka
bronchiolitis obliterans organizing pneumonia (BOOP)
COP
unknown by etiology
organizing penumonia in which all CT is of same age and no interstitial fibrosis
does not progress to honeycomb
COP symptoms
cough
dyspnea
COP Tx
oral steroid >6months
collagen vascular disorder-related lung disease associated disorder
RA sclerederma SLE sjogren syndrome polymyositis/dermatomyositis mixed CT disorder
caplan syndrome
RA and pneumoconiosis
coal dust diseases
antrhacosis
macules
progressive massive fibrosis
caplan syndroe
silica
silicosis
caplan syndrome
asbestos
asbestosis pleural plaques caplan syndrome mesothelioma carcinoma of lung, larynx, stomach, colon
beryllium
acute berylliosis
berylium granulomatosis
lung carcinoma
iron oxide
siderosis
barium sulfate
baritosis
tin oxide
stannosis
coal workers pneumoconiosis (CWP)
asymptomatic antracosis simple CSP complicated CWP pathogenesis poorly understood no increased risk of TB or CA
simple CWP
coal macules and nodules +/- centirlobular emphysema
usually benign
complicated CWP
w/progressive massive fibrosis scars >2cm pulmonary dysfunction pulmonary HTN Cor pulmonale
silicosis
currently most prevalent chronic occupational disease in the world
slowly progressive over decades
silica ingested by macros and it kills the macros
inflammation w/release of TNF -> activated fibroblasts
2x increased chance for CA
more susceptible to TB
asbestos related disease
pleural effusions pleural plaques or diffuse pleural fibrosis asbestosis lung cancer (5x increased chance) mesothlioma
asbestosis
fibrotic lung disease begins in lower lobes and subpleurally
dyspnea followed by productive cough
asbestos on histo
asbestos bodies (absent in pleural plaques) stains prussian blue b/c of iron
bleomycin
pneumonitis and fibrosis
methotrexate
hypersensitivity pneumonitis
amiodarone
pneumonitis and fibrosis
nitrofurantoin
hypersensitivity penuomnitis
aspirin
bronchospasm
beta agonists
brochospams
radiation pneumonitis
acute- 10-20% w/in 6 months of radiation
chronic- pulmonary fibrosis
sarcoidosis
numerous non-caseating granumpomas
increased CD4, IL2, and cytokines
genetic factors
diagnosis of exclusion
sarcoidosis location
can occur anywhere including CNS pulmonary >eye > skin b.l pulmonary lymphadeopathy or lung disease spleen and liver in 3/4 W>M US black >>>whites, rare in chinese
sarcoidosis recovery
65-70% full recovery
20% permanent loss of lung fnx
10-15% progress to pulmonary failure or death
hilar LN better prognosis then LN+lung infiltrates
ACE converting enzyme
elevated in: sarcoidosis leprosy gaucher disease primary biliary cirrhosis and amyloidosis
sarcoidosis histo
noncaseating sub epi granulomas
multinucleated giant cells
asteroid body
schaumann body
hypersensativity pneumonitis aka
extrinsic allergic alveolitis
farmers lung
moldy hay
micropolyspora faeni
bagassosis
moldy pressed sugar cane
thermophilic actinomycetes
humidifier lung
cool-mist humidifier
thermophilic actinomycetes
aurobasidium pullulans
pigeon breeers lung
pigeon serum proteins in droppings
interstitial lung diseases
desquamative interstitial pneumonia
respiratory bronchiolitis-associated interstitial lung disease (RBILD)
eosinopilic granuloma
DIP
large collections of smokers macrophages in alveoli minimal fibrosis >50 M>>F dyspnea steroids and cessation of smoking
RBILD
common lesion in smokers (30+pack years) pigmented macros in bronchiles peribronchiolar fibrosis usually mild may have dyspnea and cough regresses w/cessation of smoking
eosinophilic granuloma aka
pulmonary langerhans cell histocytosis
eosinophilic granuloma
proliferation of dendritic cells in response to smoking
usually polyclonal and felt to be reactive hyperplasia
regresses w/cessation of smoking
expresses CD1z, S-100, CD207
pulmonary alveolar proteinosis
uncommon
cough and sputum w/gelatinous chunks
pulmonary alveolar proteinosis histo
accumulation of acellular surfactant in intra-alveolar and bronchiolar spaces
pulmonary alveolar proteinosis classes
autoimmune/acquired
secondary
hereditary/congenital
autoimmune pulmonary alveolar proteinosis
90%
20-50
Ab to GM-CSF
recurs after transplantation
secondary pulmonary alveolar proteinosis
rare, many lung disorders, immunodeficiency syndromes, malignancy, and blood disorders
hereditary pulmonary alveolar proteinosis
rare, genetic, fatal
