Chapter 15 Robbins Flashcards
endemic area for coccidiomycosis
sw and W us and mexico
autoimmune pap
autoimmune
-abs for GM-CSF, 90% of cases, lacks familial predisp, loss of GM-CSF blocks terminal diff of alveolar macrophages impairing ability to catabolize surfactanct
legionella pneumonia common in what individuals
with predisposing conditions like cardiac, renal, imune, hematologic
focal areas of cell consoidation of lung substance chiefly in periphery of lung fields
- heavey aggregates of lymphocytes and eosinophil lesions in septal walls and alveolar spaces
- pts have cough, fever, night sweats, dyspnea, and weight loss
- response to corticosteroid therapy
idiopathic chronic eosinophilic pneumonia
adenocarcinma and genetics
GOF mutation in GF receptor signaling path
genes encoding RTKs like
-EGFR,ALK,ROS,MET,RET MR EAR
prognosis of goodpasture impvoed by
intensive plasmapheresis, also give simultaneous immunosuppressive therapy
____ pneumonia is localized lesion in ICP with or without regional LN involvement
chronic
associated with URI and LRI
most common infection in young children, elderly, and ICP
can cause bronchioloitis and pneumonia
first infection in childhood, reinfections common throughout life
clinically indistinguihsable from those caused by RSV and often mistaken for influenza
human petapneumovirus
symptom in viral laryngotracheobronchitis and bronchiolitis
vocal cord swelling and abundant mucus production
complications of lung abscess
extension into pleural cavity, hemorrhage, and development of brain abscesses or meningitis from septic emboli
what infection: associated with lung abscess and emyema
importnant cause of hospital acquired pneumonia
important cause of secondary bacterial pneumonia in children and healthy adults following viral resp illness
staphy aureus
evidence that hypersensitivity pneumonitis is immuno mediated
specimens have increased MIP1a and IL-8
- increased CD4 and CD8
- abs against causitve agent in serum
- noncaseating granulomas = t cell mediated IV hypersens
infrequent complications in lung transplant
accelerated pulmonary arteriosclerosis in the graft and EBV associated B cell lymphoma
mode of transmission legionella
inhalation of aerosolized organisms or aspiration of contaminated drinking water
PHTN clinical course
most common in women ages 20-40 and occasionally in young children
- dyspnea, fatigue, some pts have chest pain
- overtime respiratory distress, cyanosis, rvh, death from cor pulmunale within 2-5 yrs of 80% of pts
radiolograph of PAP
bilateral patchy asymmetric pulmonary opacifications
morphology of lung rejection
lymphocytes, plasma cells, few neutrophils and esoinophils around small vessels, in submucosa of airways or both
adenocarcinoma that spreads forming satellite tumors
-solitary or multiple nodule
mucinous adenocarcinomas
1 year survival rate of lung transplant
79%
during cardiopulmonary resusciation from PE pt frequently has ____
electromechanical dissociation
-ECG has rhythm but no pulses palpated bc no blood entering pulmonary arterial circulation
location of adenocarcinoma
periphery
most common bacterial cause of actute exacerbation of COPD
haem infl
surfactant protein B clinical presentation
infant is full term and rapidly develpos progressive respiratory distress shortly after birth
death btwn 3-6 months
variable amt of intra alveolar pink granular material, type II pneumocyte hyperplasia, interstitial fibrosis and alveolar simplification
abnormalities in lamerllar bodies in type II pneumocytes
surfactant dysfunction disorder morphology
treatment for human MPV
ribavirin
imaging in pulmonary langerhans cell histiocytosis
imaging shows characterisitc cystic and nodular abnormalities
cardinal histologic change in all abscesses is
suppurative destruction of lung parenchyma within the central area cavitation
etiology and pathogenesis of lung abscess
common organism are aerobic and anerobic streptococci, staph aureus, and gram negative organisms
anerobic organsism normally in oral cavity include bacteriodes, fusobacterium, and peptococcus
hereditary PAP
extremely rare
occurs in neonates caused by mutations that disrupt genes involved in GM-CSF signaling
morphology of histoplasmosis infection
granulomas with caseating necrosis and consolidation
-differentiate from TB, sarcoidosis and coccidio by identification of 3-5 micromietere yeast forms in tissues
epidemiology of goodpatrures
most cases occur in teens or 20s in male smokers
clinical features of hypersensitivity pneumonitis
acute attacks, of recurring episodes of fever dyspnea, cough, leukocytosis
- micronodular interstitial infiltrates on radiograph
- symptoms may last for 12 hours to several days
- if chronic exposure can lead to progressive resp failure
CD4+ count under 50 HIV infections
CMV, fungal, mycobacterium avium
neutrophils containing lancet shaped diplococci
streptococcus pneumoniae
squamous cell carcinoma genetics
chrom deletion involving 3p, 9p (site of CDKN2A gene) and 17p (site of TP53) gene are early events in tumor evolution
lymphangioleiomyomatosis population
primarily affects young women of childbearing age
3 types of blastomycosis
pulmonary, disseminated, and rare primary cutaneous form
bronchioloitis obliterans
major morphologic corelate of chronic rejection
occluion of small airways by fibrosis
patchy and therefore difficult to diagnose
-bronchiectasis and pulmonary fibrosis may develop in long standing cases
lobar pneumonia 4 stages of inflammatory response
CRGR
congestion
-few neutrophils, numerous bact, heavy, boggy, red lung
Red hepatization
-confluent exudation, neutrophils, red cells, fibrin fill alveolar spaces, lobe is red, firm, airless, liver consist
gray hepatization
-disintegration of red cells and persistence of fibroinosuppurative exudate = grayish brown color
resolution
what mutation is associated with pneumococcal pneumonias
mutation in MyD88 (adapter for TLRs for activation of NFkB)
chronic rejection of lung transplant occurs when and in how many pts
1/2 of pts within 3-5 yrs
DIP presentation
40s and 50s, sex equal
all pts smoke cigs
insidious onset of dyspnea and dry cough over weeks or month, with associated clubbing of digits
most common organism with health care associated pneumonia
MESR and P aeruginosa
these pts have higher mortality than those with community acquired pneumonia
pulmonary htn causes
chronic obstructive or interstitial lung diseases
antecedent congenital or acquired heart disease (MS)
recurerent thromboemboli
autoimmune disease (systemic sclerosis w/ pulmonary vasculature involvement)
obstructive sleep apnea
SARS is a ___virus that infects
coronavirus that infects LRT and spread throughout body
diagnosis of PE and dvt
pe- spiral computed tomographic angiography
DVT diagnoses with ultrasound
haem influenze with nonencapsulated forms
= nontypeable forms
spread along surface of URT and produce otitis media, sinusitis, and bronchopneumonia
neonates with prematuritiy, malignancy, and immunoddef more at risk
DIP treatment
excellent response to steroid therapy and smoking cessation
Desquamative interstitial pneumonia morphology
accumulation of large number of macrophages with dusty brown pigment in airspaces (smokers macrophages)
alveolar septa are thickened and lined by plump cuboidal pneumocytes
emphysema often present
tumors with FGFR1 amplification
squamous cell carcinoma
most common cause of death in goodpasture
uremia
lymphangioleiomyomatosis proliferation and markers
of perivascular epitheloid cells that express markers of melanocytes and SM cells
hypersensititvity pneumonitis caused by thermophilic bacteria in heated water reservoirs
humidifier or AC lung
langerhans cells positive for
S100, CD1a, CD207
pulmonary htn
defined as mean pulmonary artery pressure greater than or equal to 25mm Hg at rest
morphology of fulminant disseminated histoplasmosis
in immunosuppressed, granulomas do not form
focal accumulation of mononuclear phagocytes filled wwith fungal yeasts
CD4+ count under 200 in HIV infection
pneumocystis pneumonia
most commnly causes hostpital acauired infections, pts who are neutropenic and it has propensity to invade blood vessels with extrapulmnary spread
associated with CF and immunocomp pts
pseudomonas aeruginosa
foci of bronchopneumonia
consolidated areas of acute suppurative inflammation
- confined to one lobe but more often multilobar and bilateral and basal
- well developed lesions, granular ,gray, red, yellow
- neutrophil rich exudate
what hypersensitivity pneumonitis develops from exposure to dusts from hay that permits rapid proliferation of spores of thermophilic actinomycetes
farmers lung
5 year survival rate lung transplant
53%
where is histoplasma capsulatum endemic
mississippi rivers and caribbean
intermittent diffuse alveolar hemorrhage
young children, some adults
insidious onset of productive cough, hemoptysis and anemia
favorable response to long term immunosuppresion with prednisone and or azathioprine
idiopathic pulmonary hemosiderosis
inflammatory myofibroblastic tumor who gets it imaging microscoping mutation
children, equal M:F
imaging: single round well-defined usually peripheral mass with calcium deposits
- grayish white 3-10 cm in diameter
microscop: prolif of fibroblasts
mutation in ALK gene on chrom 2
what can show a coin lesion on an xray
histoplasmosis
control of influenza infection depends on
interfreon a and b produced by infected cells
-these upregulate expression of MX1 gene which is a GTPase that disrupts influenza gene txn
- cell mediated response, NKC
- Abs against H and N proteins
where does histoplasmosis capsulatum lung infection spread to
mediastinum
adrenals
liver
meninges
morphology of c immitis
c immitis is within macrophages or giant cells
- thick walled, nonbudding speherules 20-60 micrometers in diameter with small endospores
- pyogenic reaction superimposed when spherules rupture and release endospores
hypersensitivity pneumonitis provoked by proteins from serum, excreta, or feathers of birds
pigeon breeder’s lung
PE morphology
3/4 of in lower lobes
lines of zahn in the thrombus
hemorrhagic, raised, red blue in early stages
pleural surface covered by fibrinous exudate
-w/in 48 hrs becomes paler, then red brown as hemosiderin produced
-can have septic infarcts
CDKN2A is inactivated and its protein product P16 is lost in 65% of these tumors
squamous cell carcinoma
symptoms of respiratory bronchioloitis associated interstiial lung disease
mild with gradual onset of dyspnea and cough of smokers in 40s and 50s
-cessation results in improvement
pack yr history usually over 30
treatment for PAP
whole lung lavage and GM-CSF therapy for pts with autoimmune pap
atp binding cassete protein member 3 clinical course
pt presents first few months of life with rapid progressive respiratory failure followed by death
human metapneumovirus is a
paramyxovirus
predomiant reaction in viral infectiont
interstitial inflammatory reaction involving the walls of the alveoli
-alveoli septa are widened and edematous and usually have mononuclear inflammatory infiltrate
large collections of macrophages in airspaces in current or former smoker
desquamative intersititial pneumonia
DIP
less than 3 cm and composed entirely of dysplastic cells growing along preexisting alveolar septae
adenocarcinoma in situ
fungal infections during lung transplant mainly by
aspergillus and candida
bacterial pneumonia has 2 patterns of anatomic distribution
lobular and lobar
patchy consolidation is bronchopneumonia
consolidation of a large portion of a lobe is lobar pneumonia
chronic pneumonia is/is not granulomatous and caused by
is
M tuberculosis
histoplasma capsulatum
rare disease caused by defects related to GM-CSF or pulmonary macrophage dysfunction that results in accumulation of surfactant in intra-alveolar and bronchilar spaces
pulmonary alveolar proteinosis
HLA involved in goodpasture
HLADRB1*1501 and 1502
emboli have 2 deleterious pathophysiologic consequences
respiratory compromise due to nonperfused, although ventilated segment
hemodynamic compromise due to increased resistance to pulmonary blood flow through the lungs
complications of pneumonia
tissue destruction and necrosis = abscess formation (particualllry w/ klebsiella infection)
spread of infection to pleural cavity = empyema
bacteremic dissemination, = metastatic abscesses, endocarditis, meningitis, suppurative arthritis
abca3 mutation in surfactant disorder differentiation
small lamerllar bodies with electron dense cores
pulmonary infiltrates with noninfectious diseases in HIV
kaposi sarcoma
non hodgkin lymphoma
lung cancer
soild inhabiting dimorphic fungus
causes disease in central and SE US
blastomycosis dermatitidis
PAP presentation
adult pts present with cough and abundant sputum that often contains funks of gelatinous material
-progressive respiratory failure may occur and pts are at risk for infetion
what reactant producnts are more raised in bacterial infections than viral
CRP, procalcitonin
bacterial lower respiratory tract infections are among the most serious pulmonary disorders in HIV infections, culprits are
S pneumoniae
S aureus
H influenza
gram neg rods
PAIG pulm HIV
pulmonary eosinophilia responds to
corticosteroids
what environment does legionaella thrive in
artificial aquatic environments like water cooling towers and tubing systems of domestic water supplies
most frequent cause of gram negative bacterial pneumonia
afflicts debilitated and malnourished people particularly chronic alcoholics
klebsiella pneumoniae
makers for squamous cell carcinoma
p63 and p40
high mortality rate laryngotraheobronchitis results in airway obstruction as bronchi plugged by desne fibrin rich exudates containing neutrophils
pulmonary consolidation usually lobular and patch but can involve all of lobe
acute conjunctivitis in children
this is an infection with what
haem infl
10 year survival of lung transplant
30%
haemophilus influenzae structure
pleomorphic, gram neg, encapsulated and noncencapsulated forms
secondary PAP
uncommon
associated with hematopoietic disorders, malignancies and more
diseases impair macrophage maturation
pulmonary langerhans cell histiocytosis
cell level
mutation
accumulation of langerhans cells to form nodules along with fibroblasts, macrophages, and numerous eosinophils
some lesions behave as reactive process, others show BRAF mutations
pulmonary blastomycosis symptoms, chest xray and treatment
symptoms: fever, ab pain, night sweats, chills, anorexia, weight loss
chest x ray: lobar consolidation, perihilar infiltrates,
-upper lobes most freq involved
treat: most resolve spontaneously
diffuse infiltrates and bronchoalveolar lavage fluid contains more than 25% eosinophils
acute pulmonary eosinophilia with respiratory failure
moraxella catarrhalis
cause of bacterial pneum especially in elderly
second most common bacterial cause of acute exacerbation of COPD
-1 of 3 most common causes of otitis media in chidlren
chest radiograph and PE
12-36 hours after occurance may find a wedge shaped infiltrate
CD4+ count over 200 in HIV and infection
TB and bacterial infections
microinvasive adenomcarcinoma
-outcome?
tumors that are 3 cm or less with small invasive component associated with scarring and peripheral lepididc growth pattern
-better outcome than invasive carcinoma of same size
surfactant protein C
second most common of 3 surfactant disorders
hospital acquired pneumonia
pts on mechanial ventilation are at particularly high risk
gram positive S aureus and S pneumonia and gram negative rods (enterobacteriaceae and pseudomonas)
surfactant dysfunction disorders
ATP binding cassete protein member 3 (AR)
surfactant protein C (AD)
surfactant protein B (AR)
PHTN morphology
medial hypertrophy of pulmonary muscular and elastic arteries, pulmonary arterial atherosclerosis and RVH
arterioles and small arteries are most prominently affected by striking medial hypertrophy and intimal fibrosis
extreme is plexiform lesion, spans lumens of dilated thin walled small arteries and may extend outside vessel
- prominent in idiopathic and familial phtn group 1
- also unrepaired congenital heart disease with L-R shunts (group 2)
- pulmonary htn associated with HIV infection
causes legionaires disease and pontiac fevere
legionaella pneumophila
pts in pulmonary langerhans cell histiocytosis
usually young smokers
polyangiitis with granulomatosis diagnosis
transbronchial lung biopsy
capillartiis, scattered, poorly formed granulomas
3 types of pap
autoimmune
secondary
hereditary
respiratory bronchiolitis associated interstitial lung disease
chronic inflammation and peribronchiolar fibrosis
common in cig smokers
pigmented intraluminal macrophages in respiratory bronchioles
symptoms of coccidio infection
most asymptomatic
10% get lung lesions, fever, cough, pleuritic pains with erythema nodusum or erythema multiforme
less than 1% get C immitis infection which involves skin and meninges
frequent cause of death in aspiration pneumonia and organisms recovered
necrotizing pneumonia
more than one organism, most often aerobes
-abscesses
treatment for PHTN
vasodilators for group 1 or refractory disease belonging to other groups
idiotpathic pulmonary arterial HTN
encountered in pts whom all known causes are excluded
- 80% have genetic basis inherited in AD trait
- BMPR2 gene (TGF-B family)
- haploinsufficiecny leads to dysfunction and proiferation of endothelial cells and vascular SM cells
- 2 hit model like RB
encapsulated forms of haemophilus influenzae
a-f
b most virulant
3 most common causes of otitis media in children
s pneumoniae, h influenza, m catarrhalis
location of localized lung disease with histoplasmoisis
lung apices
spectrum of immune mediated predominantly interstitial lung disorders caused by intense often prolonged exposure to inhaled organic antigens
hypersensitivity pneumonitis
morphology box for pulmonary alveolar proteinosis
homogenous, granular precipitate containing surfactant proteins within alveoli
-causes focal to confluent consolidation of large area of lung with minimal inflamation
alveoli filled with granular pink precipitate
-marked increase in size and weight of lung
periodic acid schiff positive, contains cholesterol clefts and surfactant proteins
______ infectivity is bc infective arthroconidia when ingested by alveolar macrophages block fusion of phagosome and lysosome so resist intracellular killling
coccidiodomycosis immitis
lymphangioleiomyomatosis presentation and treatment
dyspnea and spontaneous pneumothorax
need lung transplant
what pts are most susceptible to histoplasmosis infection
immunocompromised
highest frequency of TP53 mutations of all lung carcinomas is
squamous cell carcinoma
diagnosing legionella
culture is gold standard
cell mediated immune defects increases pulmonary infections with
mycobacteria, herpres, and pneumocystis jiroveci
morphology of hypersensitivity pneumonitits
histologic changes centered on bronchioles
-interstitial pneumonitis, consisting primarily of lymphocytes, plasma cells, and macrophages (eosinophils rare)
- noncaseating granulomas in 2/3 of pts
- interstitial fibrosis with fibroblastic foci, honeycombing, and obliterative bronchiolitis
- intraalveolar infiltrate in over half pts
what chromosomes are associated with hamartoma
6 and 12
lymphangioleiomyomatosis mutation
TSC2, linked to tuberus scleoris (tuberin)
-mutation increases mTOR activity
which bacterial superinfection on top of influenza virus can produce ARDS and be life threatening
staph aureus
organisms are introduced in lung abscesses the following ways
MAANS in (LA)
miscellanesous
direct traumatic penetrations of lungs, spread from neighbor organ, hematogen seeding of lung-
aspiration
antecedent primary lung infection: S aureus, S pneumoniae
neoplasm
Septic emboli
small cell carcinoma genetics
loss of TP53 in 75-90% and loss of RB in almost 100%
amplification of MYC family
respiratory bronchioloitis associated interstitial lung disease
changes are patchy with bronchiolocentric distribution
dusty brown smoker macrophages similar to those seen in DIP
what is being described: small lesion less than or equal to 5mm characterized by dysplastic pneumocytes lining alveolar walls that are mildly fibrotic
atypical adenomatous hyperplasia
