Chapter 15 Robbins Flashcards
1
Q
endemic area for coccidiomycosis
A
sw and W us and mexico
2
Q
autoimmune pap
A
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
3
Q
legionella pneumonia common in what individuals
A
with predisposing conditions like cardiac, renal, imune, hematologic
4
Q
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
A
idiopathic chronic eosinophilic pneumonia
5
Q
adenocarcinma and genetics
A
GOF mutation in GF receptor signaling path
genes encoding RTKs like
-EGFR,ALK,ROS,MET,RET MR EAR
6
Q
prognosis of goodpasture impvoed by
A
intensive plasmapheresis, also give simultaneous immunosuppressive therapy
7
Q
____ pneumonia is localized lesion in ICP with or without regional LN involvement
A
chronic
8
Q
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
A
human petapneumovirus
9
Q
symptom in viral laryngotracheobronchitis and bronchiolitis
A
vocal cord swelling and abundant mucus production
10
Q
complications of lung abscess
A
extension into pleural cavity, hemorrhage, and development of brain abscesses or meningitis from septic emboli
11
Q
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
A
staphy aureus
12
Q
evidence that hypersensitivity pneumonitis is immuno mediated
A
specimens have increased MIP1a and IL-8
- increased CD4 and CD8
- abs against causitve agent in serum
- noncaseating granulomas = t cell mediated IV hypersens
13
Q
infrequent complications in lung transplant
A
accelerated pulmonary arteriosclerosis in the graft and EBV associated B cell lymphoma
14
Q
mode of transmission legionella
A
inhalation of aerosolized organisms or aspiration of contaminated drinking water
15
Q
PHTN clinical course
A
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
16
Q
radiolograph of PAP
A
bilateral patchy asymmetric pulmonary opacifications
17
Q
morphology of lung rejection
A
lymphocytes, plasma cells, few neutrophils and esoinophils around small vessels, in submucosa of airways or both
18
Q
adenocarcinoma that spreads forming satellite tumors
-solitary or multiple nodule
A
mucinous adenocarcinomas
19
Q
1 year survival rate of lung transplant
A
79%
20
Q
during cardiopulmonary resusciation from PE pt frequently has ____
A
electromechanical dissociation
-ECG has rhythm but no pulses palpated bc no blood entering pulmonary arterial circulation
21
Q
location of adenocarcinoma
A
periphery
22
Q
most common bacterial cause of actute exacerbation of COPD
A
haem infl
23
Q
surfactant protein B clinical presentation
A
infant is full term and rapidly develpos progressive respiratory distress shortly after birth
death btwn 3-6 months
24
Q
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
A
surfactant dysfunction disorder morphology
25
treatment for human MPV
ribavirin
26
imaging in pulmonary langerhans cell histiocytosis
imaging shows characterisitc cystic and nodular abnormalities
27
cardinal histologic change in all abscesses is
suppurative destruction of lung parenchyma within the central area cavitation
28
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
29
hereditary PAP
extremely rare
| occurs in neonates caused by mutations that disrupt genes involved in GM-CSF signaling
30
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
31
epidemiology of goodpatrures
most cases occur in teens or 20s in male smokers
32
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
33
CD4+ count under 50 HIV infections
CMV, fungal, mycobacterium avium
34
neutrophils containing lancet shaped diplococci
streptococcus pneumoniae
35
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
36
lymphangioleiomyomatosis population
primarily affects young women of childbearing age
37
3 types of blastomycosis
pulmonary, disseminated, and rare primary cutaneous form
38
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
39
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
40
what mutation is associated with pneumococcal pneumonias
mutation in MyD88 (adapter for TLRs for activation of NFkB)
41
chronic rejection of lung transplant occurs when and in how many pts
1/2 of pts within 3-5 yrs
42
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
43
most common organism with health care associated pneumonia
MESR and P aeruginosa
| these pts have higher mortality than those with community acquired pneumonia
44
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
45
SARS is a ___virus that infects
coronavirus that infects LRT and spread throughout body
46
diagnosis of PE and dvt
pe- spiral computed tomographic angiography
DVT diagnoses with ultrasound
47
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
48
DIP treatment
excellent response to steroid therapy and smoking cessation
49
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
50
tumors with FGFR1 amplification
squamous cell carcinoma
51
most common cause of death in goodpasture
uremia
52
lymphangioleiomyomatosis proliferation and markers
of perivascular epitheloid cells that express markers of melanocytes and SM cells
53
hypersensititvity pneumonitis caused by thermophilic bacteria in heated water reservoirs
humidifier or AC lung
54
langerhans cells positive for
S100, CD1a, CD207
55
pulmonary htn
defined as mean pulmonary artery pressure greater than or equal to 25mm Hg at rest
56
morphology of fulminant disseminated histoplasmosis
in immunosuppressed, granulomas do not form
| focal accumulation of mononuclear phagocytes filled wwith fungal yeasts
57
CD4+ count under 200 in HIV infection
pneumocystis pneumonia
58
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
59
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
60
what hypersensitivity pneumonitis develops from exposure to dusts from hay that permits rapid proliferation of spores of thermophilic actinomycetes
farmers lung
61
5 year survival rate lung transplant
53%
62
where is histoplasma capsulatum endemic
mississippi rivers and caribbean
63
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
64
```
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
65
what can show a coin lesion on an xray
histoplasmosis
66
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
67
where does histoplasmosis capsulatum lung infection spread to
mediastinum
adrenals
liver
meninges
68
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
69
hypersensitivity pneumonitis provoked by proteins from serum, excreta, or feathers of birds
pigeon breeder's lung
70
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
71
CDKN2A is inactivated and its protein product P16 is lost in 65% of these tumors
squamous cell carcinoma
72
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
73
treatment for PAP
whole lung lavage and GM-CSF therapy for pts with autoimmune pap
74
atp binding cassete protein member 3 clinical course
pt presents first few months of life with rapid progressive respiratory failure followed by death
75
human metapneumovirus is a
paramyxovirus
76
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
77
large collections of macrophages in airspaces in current or former smoker
desquamative intersititial pneumonia
| DIP
78
less than 3 cm and composed entirely of dysplastic cells growing along preexisting alveolar septae
adenocarcinoma in situ
79
fungal infections during lung transplant mainly by
aspergillus and candida
80
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
81
chronic pneumonia is/is not granulomatous and caused by
is
M tuberculosis
histoplasma capsulatum
82
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
83
HLA involved in goodpasture
HLADRB1*1501 and 1502
84
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
85
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
86
abca3 mutation in surfactant disorder differentiation
small lamerllar bodies with electron dense cores
87
pulmonary infiltrates with noninfectious diseases in HIV
kaposi sarcoma
non hodgkin lymphoma
lung cancer
88
soild inhabiting dimorphic fungus
| causes disease in central and SE US
blastomycosis dermatitidis
89
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
90
what reactant producnts are more raised in bacterial infections than viral
CRP, procalcitonin
91
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
92
pulmonary eosinophilia responds to
corticosteroids
93
what environment does legionaella thrive in
artificial aquatic environments like water cooling towers and tubing systems of domestic water supplies
94
most frequent cause of gram negative bacterial pneumonia
afflicts debilitated and malnourished people particularly chronic alcoholics
klebsiella pneumoniae
95
makers for squamous cell carcinoma
p63 and p40
96
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
97
10 year survival of lung transplant
30%
98
haemophilus influenzae structure
pleomorphic, gram neg, encapsulated and noncencapsulated forms
99
secondary PAP
uncommon
associated with hematopoietic disorders, malignancies and more
diseases impair macrophage maturation
100
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
101
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
102
diffuse infiltrates and bronchoalveolar lavage fluid contains more than 25% eosinophils
acute pulmonary eosinophilia with respiratory failure
103
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
104
chest radiograph and PE
12-36 hours after occurance may find a wedge shaped infiltrate
105
CD4+ count over 200 in HIV and infection
TB and bacterial infections
106
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
107
surfactant protein C
second most common of 3 surfactant disorders
108
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)
109
surfactant dysfunction disorders
ATP binding cassete protein member 3 (AR)
surfactant protein C (AD)
surfactant protein B (AR)
110
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
111
causes legionaires disease and pontiac fevere
legionaella pneumophila
112
pts in pulmonary langerhans cell histiocytosis
usually young smokers
113
polyangiitis with granulomatosis diagnosis
transbronchial lung biopsy
| capillartiis, scattered, poorly formed granulomas
114
3 types of pap
autoimmune
secondary
hereditary
115
respiratory bronchiolitis associated interstitial lung disease
chronic inflammation and peribronchiolar fibrosis
common in cig smokers
pigmented intraluminal macrophages in respiratory bronchioles
116
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
117
frequent cause of death in aspiration pneumonia and organisms recovered
necrotizing pneumonia
more than one organism, most often aerobes
-abscesses
118
treatment for PHTN
vasodilators for group 1 or refractory disease belonging to other groups
119
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
120
encapsulated forms of haemophilus influenzae
a-f
| b most virulant
121
3 most common causes of otitis media in children
s pneumoniae, h influenza, m catarrhalis
122
location of localized lung disease with histoplasmoisis
lung apices
123
spectrum of immune mediated predominantly interstitial lung disorders caused by intense often prolonged exposure to inhaled organic antigens
hypersensitivity pneumonitis
124
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
125
______ infectivity is bc infective arthroconidia when ingested by alveolar macrophages block fusion of phagosome and lysosome so resist intracellular killling
coccidiodomycosis immitis
126
lymphangioleiomyomatosis presentation and treatment
dyspnea and spontaneous pneumothorax
| need lung transplant
127
what pts are most susceptible to histoplasmosis infection
immunocompromised
128
highest frequency of TP53 mutations of all lung carcinomas is
squamous cell carcinoma
129
diagnosing legionella
culture is gold standard
130
cell mediated immune defects increases pulmonary infections with
mycobacteria, herpres, and pneumocystis jiroveci
131
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
132
what chromosomes are associated with hamartoma
6 and 12
133
lymphangioleiomyomatosis mutation
TSC2, linked to tuberus scleoris (tuberin)
| -mutation increases mTOR activity
134
which bacterial superinfection on top of influenza virus can produce ARDS and be life threatening
staph aureus
135
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
136
small cell carcinoma genetics
loss of TP53 in 75-90% and loss of RB in almost 100%
| amplification of MYC family
137
respiratory bronchioloitis associated interstitial lung disease
changes are patchy with bronchiolocentric distribution
| dusty brown smoker macrophages similar to those seen in DIP
138
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
