Nuggets Flashcards
what is CTLA-4/what does it do?
-receptor on activated T cells and regulatory T cells
-prevents further activation by competing with activating receptor CD28 (for ligands B7-1/CD80 and B7-2/CD86)
which adhesion molecule mediates FIRM adhesion of leukocytes in blood vessels?
integrins
- leukocyte function-association antigen (LFA-1) - macrophage antigen-1 (MAC-1)
these bind to ICAM-1 and 2 on endothelium
in the MHC 1 pathway, what protein mediates transport of peptides from cytosol to the ER?
TAP protein
Which T cell ligand-receptor interaction:
1) activates naive T cells
2) activates and maintains effector and memory T cells?
1) naive: CD28 + ligands CD80/B7-1, CD86/B7-2
2) effector/mem: ICOS receptor + ICOS ligand
which types of HAE have normal C1 inhibitor?
Factor XII mutation
ANGPT1 mutation (angiopoietin-1) not related to bradykinin pathway
plasminogen mutation
what is the p-i concept?
-pharmacologic interaction of drugs with immune receptors concept
-drug hypersensitivity mechanism - medications directly interacts with the MHC or TCR molecules to stimulate an immune response
-e.g. abacavir with HLA-B*5701
Which Fc Receptor expressed on the natural killer (NK) cell is responsible for antibody-dependent cell-mediated cytotoxicity (ADCC)?
FcyRIIIA
contraindications to bronchial thermoplasty
implantable electronic devices
sensitization to meds needed for procedure
previous treatment in same area
active resp infection
active asthma exacerbation or OCS dose change in let 14 days
known coagulopathy
During an immune response, which chemokine promotes eosinophil recruitment and infiltration into tissues?
CCL11
what lipid mediators are newly synthesized in mast cells when activated?
prostaglandin D2
LTB4, LTC4, LTD4, LTE4
Which mediator is a neutrophil chemokine that promotes MIGRATION?
CXCL8 aka IL8
transcription factor associated with Th2 cells?
STAT6
GATA3
Which chemokine receptor is associated with eosinophil, basophil and mast recruitment?
CCR3
main cause of symptoms of nasal congestion
interaction between airflow and airflow RECEPTORS in nasal mucosa
Which cytokine is most responsible for pruritus in atopic dermatitis?
IL-31
what do you check nasal discharge for to check if it’s CSF fluid?
beta-2 transferrin
vernal keratoconjunctivitis highlights
warm, dry climates, warm seasons
young atopic males
thick discharge/pain/photophobia
giant papillae >1mm, honer truants dots (per limbic white dots) - only conjunctivae and cornea affected
which heterozygous C’ deficiency causes mildly low CH50?
hetero C2 deficiency
nonbronchodilator effects of beta agonist (7)
- Decreased microvascular permeability
- Inhibit cholinergic neurotransmission
- Inhibit mediator release from basophils, mast cells, and lung tissue
- Priming glucocorticoid receptor activity
- Increase mucociliary clearance
- Protect epithelium from bacteria
- Inhibit function of bronchial epithelial cells, eosinophils, macrophages and T cells
pretreatment prior to ASA desens in AERD?
leukotriene modifying drug
Which of the following measures of lung function is most useful at monitoring adherence and predicting exacerbations?
FeNO
most common genetic cause of primary ciliary dyskinesia?
DNAH5 mutation
pork cat syndrome
pts sensitized to FelD2 react to ingested pork within 30-45 min
GI sxs common, but can be anything up to anaphylaxis
mutation in type 2 HAE?
missense SERPING1 gene
what class of drugs increases risk of ACEi induced angioedema?
DPPV-4 inhibitors (gliptins/liptins)
immunosuppressive drugs
first line HAE ppx in pregnancy?
pdC1-INH (subQ)
first line HAE ppx in peds?
pdC1-INH (subQ every 3-4days)
Alternative in 12+yo: lanadelumab (subQ every 2-4 wks)
which HAE abortive therapy can be self-administered at home?
icatibant
what HAE medication has some rabbit components?
recombinant C1-INH
how long to delay new treatment after ACEi induced angioedema?
6 weeks
which erythrocyte bound receptor results in decreased clearance of immune complexes from circulation?
CR1/CD35 (complement receptor 1)
which IgG doesn’t participate in complement activation?
IgG4
region along the precipitin curve, which results in the most pathogenic Ab/Ag immune complexes
zone of moderate antigen excess
what product/molecule is found in conjunctivae and corneal pathology of vernal keratoconjunctivitis?
conjunctivae: eosinophil major basic protein
cornea: eosinophil peroxidase, eosinophil cationic protein
MOST common corneal pathology seen in atopic keatoconjunctivitis?
punctate epithelial keratopathy
less common: corneal scarring, keratoconus, shield ulcers
which med for ankylosing spondylitis also has specific indication for management of uveitis?
infliximab
peripheral ulcerative keratitis sxs/s, dz assn, tx
s/s: few days of unilateral progressively worsening ocular pain, light sensitivity, redness, tearing, yellow-white peripheral corneal infiltrates
dz assn: s aureus blepharitis
tx: abx + topical steroids
cytokines involved in pathogenesis of irritant contact dermatitis?
**IL1alpha **
IL1beta, TNFalpha
what molecule is increased in tears of pt with giant papillary conjunctivitis?
tryptase
how do maternal smoking and gender influence AR risk
smoking: increase
male: increase
anhydrides are LOW or HIGH molecular weight and found in __?
LOW molecular weight
found in paints, varnishes, plastics
what receptor is activated in irritant rhinitis?
TRPA1 (transient receptor potential ankyrin type 1)
where are the highest eos counts in the GI tract (normally)?
colon
which first line EOE tx has 75% success rate after 8 weeks?
6 food elimination
which cytokine is most important for terminal differentiation of eos?
IL5
SPT vs sIgE sens and spec
SPT more sensitive
sIgE more specific
when do you SPT in occupational asthma
for high molecular weight sensitizers
and certain low molecular weight sensitizers (acid anhydride, sulfonechloramide, vinyl sulfone reactive dyes, per sulfate salts, platinum salts)
lab findings in wiskott Aldrich syndrome
low and small plt (normal megakaryocytes)
which C’ deficiency is ass’d with SLE?
C2 deficiency
IRAK4 and MyD88 deficiency main characteristics
- Defect in TLR signaling
- Pyogenic bacterial infections pneumococcal, pseudomonas without fever
- Improve with age
- AR inheritance
Types of T-B+NK- SCID
- JACK3 deficiency
- Xlinked SCID
what markers can be used to determine basophil activation?
CD63 and CD203c
what innate immunity associated receptors do basophils express?
TLR
leukocyte Ig-like receptors (LIR)
mediators that mast cells produce but basophils do not (or very little) (6)
tryptase
chymase
carboxypeptidase
heparin
PGD2
LTB4
transcription factors for eos
GATA-1
PU.1
C/EBP members
which cytokines promote maturation of eos from myeloid precursors? (3)
GMCSF
IL3
IL5
which cell is the body’s major producer of type I IFNs
plasmacytoid dendritic cells
important for innate immune response to viruses
what cytokine targets immature lymphoid progenitors to induce T lymphs?
IL7
which chemokine receptor is expressed by activated DCs?
CCR7 (also expressed on naive T cells)
impact of puberty, menses, pregnancy on nasal airflow?
vasodilation + glandular hypersecretion -> decreased flow
molecules expressed on T cells infiltrating skin (e.g. atopic derm) (4)
CLA
CCR4
CCR10
CRTH2
what is methylisothaizoline in and where is it’s classic contact derm rash?
sunscreen, cosmetics, wipes, hair products, liquid soap, laundry products
face, hands, buttocks
what is reactive airways dysfunction syndrome?
type of occupational asthma
aka irritant induced asthma
labs findings in HP
BAL: CD4:CD8 <1
lymphocytosis >20%
elevated PMNs, eos, mast cells, Igs, hyaluronic acid
biopsy: noncaseating granulomas, mononuclear cell infiltrates
meds for idiopathic pulm fibrosis
nintedanib
pirfenidone
basics of exercise challenge test
2-3min warm up to reach target HR of 80-90% max HR
last 6 min in kids, 8 min in adults
sxs and gene of mevalonate kinase deficiency aka hyperIgD syndrome
MVK
periodic fevers, LAD, rash, oral ulcers, rash, GI sxs, splenomegaly autoinflammatory dz
triggers: illness, vaccinations
genes for T-B-NK- SCID (3)
ADA
RAC2
AK2
NEMO deficiency gene and s/s
IKBKG (NF kappa B essential modulator)
ectodermal dysplasia, infections - mycoplasma, pneumococcal, staph
WHIM syndrome s/s and gene
warts, hypogamm (can happen later), infections, myelokathexis (causes neutropenia bc can’t exit BM), T and B lymphopenia
GOF variant in CXCR4
which C’ deficiencies have normal CH50 but absent AH50
factor B, factor D, properdin
what is autophagy?
autophagosomes engulf cytosolic proteins (intracellular) and fuse with lysosome to enter the MHC II pathway to be presented to CD4+ T helper cells
what receptor expression is increased with IVIG and sends inhibitory signals to B cells?
FcyRIIB
what does activation of IL2 receptor gamma lead to? (immediately)
STAT5 phosphorylation
what is Siglec-8 and where is it expressed
inhibitory receptor
eos, mast cells > basophils
LAD type II s/s and gene
poor but not absent pus formation, Recurrent skin infections, microcephaly, developmental delay, short stature, periodontal infections and Bombay blood group (absence of H antigen, hh phenotype) delayed umbilical cord separation is not observed.
Mutations in SLC35C1 (encodes GDP-fucose transporter 1 (FUCT1) and leads to the absence of fucosylated carbohydrate ligands and absence of Sialyl-LewisX (sLeX) expression (CD15a))
cell surface markers on naive T cells
CD45A
L selection (CD62L)
CCR7
type I interferon function
(IFN alpha and beta)
key cytokine in antiviral response
induce gene expression that viral transcription, degrade viral RNA via RNases, and inhibit viral protein synthesis
sequester lymphs in lymphoid tissues
surface markers of effector T cell
CD25
CD45RO
what type of B cell has high rate of somatic hypermutation?
follicular B2 cells
staining for what can ID macrophages (e.g. Mac activation in HLH in BM)
CD163
NNT = ?
NNT = 1/ARR
ARR = control event rate - treatment event rate
which antihistamines don’t require adjustment for renal impairment
first gen
loratidine
most common lab technique to measure Igs
nephelometry
which proliferation assay gives % of proliferating cells with fluorescent dye dilution
carboxyfluorescein succinimidyl ester (CFSE) assay
what med affects results of BAT?
oral steroids
what is the major allergen of honeybee
phospholipase A2
which segment of the Ig is most specific binding site for 3D epitopes
complementary determining region = hypervariable region
how many globular domains in each types of Ig
4 in IgG, IgD, IgA
5 in IgM, IgE
What structural component of an immunoglobulin distinguishes isotypes from one another?
Fc portion (changes what they bind to and their effector functions)
proposed mechanisms of DRESS
drug detox enzyme abnormalities with accumulation of reactive frug metabolites
sequential reactivation of herpesviruses
HLA allele genetic predispositions
BAL and biopsy in sarcoidosis
BAL showed increased lymph and Mac, increased CD4/CD8 ratio
biopsy showed noncaseating granuloma
XMEN (x linked immunodeficiency magnesium defects-EBV infection-neoplasia) s/s and genetics
mutation in Mag transporter protein MAGT1
uncontrolled EBV viremia, lymphoma, cutaneous HPV infections
what is affected in X linked T-B+NK- SCID
common gamma chain (CD132) of IL2 receptor gene IL2RG
deletion of __ in the T lineage results in loss of Treg IPEX syndrome
dicer (endoribonuclease)
Omenn syndrome mutations and affected process/enzyme
RAG1/2 - affects VDJ recombinase
ARTEMIS - affected hairpin opening, end processing and joining steps of VDJ recombination
CD4:CD8 ratio normal and during acute infection
normal - 2:1
acute infection - 1:25
most important cytokine for maintenance of memory T cells
IL7
IL15
What is the underlying mechanism of cytotoxic T cell (CTL) exhaustion in chronic viral infections/tumors
expression of inhibitory receptors (PD1, CTLA4)
Which adhesion molecules are associated with CTL-mediated target cell killing
integrin LFA-1 on CTL binds to ligand ICAM-1 on target cell
T-independent cell-mediated immunity process is facilitated by __ activation
TLR activation
what is the ligand for CCR9 and where is it found
ligand = CCL25
found in gut epithelium, facilitates gut homing of activated lymphocytes
which HLA genotypes ahve highest risk of celiac disease
HLADQ2 and DQ8
what induces CCR10 expression in the skin
AND
ligand for CCR10
Vit D binding to vit D receptor in effector T cells
CCR10 goes on to recognize its ligand CCL27 made by keratinocytes
immune regulator proteins of airway
surfactant proteins A and D
typical order of development of AERD
- CRSwNP
- asthma
- ASA hypersens
target dose of ASA desens for different indications
- CV dz prevention
- maintain cross-desens to all NSAIDs
- AERD
- CV dz prevention: 81mg
- maintain cross-desens to all NSAIDs: 325 mg
- AERD: 650mg BID
How long does it typically take for cow’s milk and soy protein-induced proctocolitis to resolve after allergen avoidance?
6mth-2yr
APS-1 (autoimmune polyendocrine syndrome type 1) basics
autoimmunity of endocrine organs + thrush
mutation in AIRE gene = failure of central tolerance
autoAb to IL-17 = susceptible to mucocut candidiasis
CTLA-4 haploinsufficiency vs LRBA deficiency
CTLA-4 haploinsufficiency
- recurrent inf, hypogamm, lymphocytic infiltration into organs including lungs
LRBA deficiency
- AR
- LRBA is involved in endocytosis/recycling of CTLA-4
- mutations cause similar lymphoproliferative syndrome as CTLA-4
IL10 deficiency
severe colitis before 1yo
decreased IL10 –> increased mac and DC activation –> uncontrolled lymph and mac activation –> increase MHC class II, IL12, and IFN gamma
what does IL10 do
inhibits production of IL12 and class II MHC molecules by DC and macrophages
inhibits IFN gamma production
what is CD95
death receptor FAS
what is epitope spreading
immune response is initiated against a previously hidden self-antigen. The injury to tissues results in further release of other antigens with activation of lymphocytes specific to the other antigens. This leads to disease exacerbation
which HLA is ass’d with ankylosing spongylitis>
HLA B 2705
what mutations are ass’d with SLE
FcyRIIB
HLA DRB1-1501 and 0301
functions of IL2 (5)
potent activator of the proliferation and function of T lymphocytes and natural killer cells
- T cell growth factor
-can augment natural killer (NK) cell cytolytic activity
-contributes to the development of (Treg) cells
-promotes immunoglobulin production by B cells
-regulates the expansion and apoptosis of activated T cells
Which cytokine modulates eosinophilic production, maturation, activation, and survival in blood
IL5
IgE binds to which subunit of FcepislonRI?
alpha
The Early Asthmatic Reaction is a decline in respiratory function within how many minutes ofbronchial allergen challenge?
10-20min
The percentage of which type of mast cell is increased in the skin of patients with atopic dermatitis?
MCt
normal skin is 90% MCtc
AERD urinary measurement?
increased urinary LTE4 (reflects increased production of cysLTs)
Which cytokine plays a significant role in the survival and activation of mature basophils?
IL3
basophils have high expression of IL3R (CD123)
If there is an exposure to an allergen, the bone marrow activates and increasesthe production of the following cells:
eos
basophils
Which cytokines inhibits the priming effects that IL-3 has on basophil function?
IFN alpha
IFN beta
How do basophils provide signals for B cells to produce IgE?
Expressing CD40L and secreting IL-4 and IL-13
Committed eosinophil precursors in the bone marrow can be identified by their expression of?
IL5R (CD125)
CCR3
which eos granule [ ] in BAL fluid is correlated to bronchial hyperactivity in asthmatics?
major basic protein
predominant metabolic of 5 lioxygenase pathway in eos?
LTC4 (then metabolized to LTD4 and LTE4)
eos derived substances and their effects in pathogenesis of asthma
MBP/eos cat protein, EDN, EPX
MMP9
LTC4
TGFbeta
MBP/eos cat protein, EDN, EPX - epithelial injury
MMP9 - tissue damage and remodeling
LTC4 - smooth muscle contraction
TGFbeta - tissue remodeling and fibrosis
cytokines released by Th17
IL17
IL22
What is the primary determinant of the site of lung deposition, distribution of the drug within the lung, and resulting deposition efficiency?
size of aerosol particle
most ultrasonic nebulizers operate at what frequency
> 1MHz
Which HFA inhaled corticosteroid has a built-in spacer
flunisolide
For patients with a history of severe anaphylaxis, what should be the initial dose for drug desensitization?
between 1/10,000 and 1/1,000,000
In the airway epithelial cells, glucocorticoids suppress the expression and release of a number of inflammatory cytokines, chemokines, and growth factors likely by affecting which transcription factor?
NFkB
Inhibitors targeted at which enzyme have been able to restore glucocorticoid sensitivity inpatients with steroid refractory asthma?
P38 MAPK (MAPK14)
Which interleukin, associated with neutrophilic asthma, is elevated in sputum and serum samples from patients with severe asthma and has been shown experimentally to inhibit corticosteroid responsiveness in human bronchial epithelial cells?
IL17
The primary toxicity associated with zafirlukast and zileuton affect which organ?
liver - elevated LFTs
Which protein helps activated naïve T-cells remain in the lymph node?
CD69 - protein that binds S1PR1 and reduces its cell surface expression –> prevents naive T cell from migrating out of LN into blood (where [S1P] is higher)
What do bone marrow-homing IgG-secreting plasma cells express? (2)
VLA4 and CXCR4
Genetic deficiency in UNC-93B results in susceptibility to:
HSV infections or other DNA/RNA viruses
protein on ER required for endosomal localization and function of TLR 3, 7, 8, 9
Type 1 interferons (IFN-α and IFN-β) actions? (5)
protect against viral infections by:
1) requesting lymphs in LN
2) up regulating class I MHC
3) increasing cytotoxicity of NK cells
4) promoting differentiation of naive T cells to Th1 cells
avidity vs affinity
Avidity is a semiquantitative term used to describe the overall binding of antibodies to antigen at all antigen binding sites.
Affinity refers to the strength of a single antigen-antibody interaction.
what Ab do immature B cells produce
membrane IgM
what type of residue do m membrane forms of Ig heavy chains have
hydrophilic amino acid residues but hydrophobic transmembrane portion
cytoplasmic domains differ between isotypes
where is the hinge region of the Ab located and what it is purpose
between CH1 and CH2
flexibility permits independent movement of antigen binding sites
protein digestion in MCH I vs II pathway
MHC I: occurs in proteasome
MHC II: occurs in phagolysosome
Which type of cellular receptor is an integral membrane protein with intrinsic catalytic enzyme domain(s) inthe cytoplasmic tail?
receptor tyrosine kinase
What are the main ligands for the IL-1 receptor family?
IL1
IL18
both are involved in the activity of inflammasome
JAK2 GOF disease
myelodysplastic syndromes
which Ab are transferred to infants via breastmilk
IgG and IgA
Which cytokine increases eosinophil apoptosis
IL4 (in contrast to most other Th2 cytokines)
Activation of which receptors leads to eosinophil apoptosis?
FAS ligand receptor
The 5-lipoxygenase pathway is most abundant in which cell type:
myeloid
In granulomatosis with polyangiitis, which types of cytokines are increased in response to environmental stimuli and contribute to granuloma formation?
Th1/Th17 cytokines (IL17, TNF, IFN gamma)
most common extrapulm manifestations of EGPA
mononeuritis multiplex
what contributes to fibrosis in sarcoidosis?
down regulation of Th1 response (increased in initial phase of sarcoidosis)
release of TGFB and CCL10
Mutations in the gene that encodes which chemokine receptor protects human cells from HIV infection?
CCR5
What medications have been implicated in causing eosinophilia and pneumonitis
nitrofurantoin
semi-synthetic PCN
NSAIDs
What is the defect in MHC class II deficiency?
Class II transactivator (transcriptional activator of MHC class II genes)
deficiency of RFX complex (binds to promotor of MHC class II genes)
What contributes to the heterogeneity of MHC? (3)
1) replacement substations are more common than silent mutation
2) MHC molecules are codominants and alleles are expressed equally int he cells
3) products of individual MHC alleles/isoforms can differ from one another by up to 20 AAs, making each variant protein distinct
transcription factor that is very important for dendritic cell development and for cross-presentation?
CXCR1 - uniquely expressed by DC subset that acts in cross presentation
super antigens lead to massive production of cytokines by what cells
CD4 cells
Differentiation of the multipotent progenitor cell into the common lymphoid progenitor requires signaling through which receptor?
FLT3 receptor bindings to its FLT3 ligand
During which stage of B cell development, does the heavy chain rearrangement begin?
early pro-B cell stage
The process of isotypic exclusion is:
Expression of only 1 of 2 light chains in a B cell, such that kappa chains are rearranged first and if unsuccessful, are followed by lambda chains.
Structure of the pre-TCR is
BETA chain then pairs with pTalpha chain
alpha chain locus is rearranged in what stage of T cell development
double positive stage
the pre TCR signals constitutively via what protein
Lck
what shuts off V alpha chain rearrangement in cells
recognition of self MHC: self peptide complex which positively selects the receptor
What cytokine in required for Treg development?
IL2
Which of B cell maturation steps is sometimes impaired in ataxia telangiectasia due to a mutation in the ATM gene?
class switching
B cell coreceptor complex contains which cell surface proteins
CD19
CD21 (aka CR2)
CD81
on what chromo is the locus encoding the k light chain gene
chromo 2
what is VDJ recombination guided by
recombination signal sequences
(noncoding DNA sequences, found adjacent to point at which recombination takes place)
what segments do Ig heavy vs light chain and TCR alpha vs beta have
Ig heavy chain and TCRbeta have VDJ
Ig light chain and TCRalpha have VJ
TCR have more J gene segments and there is greater diversification of the junction between gene segments during the process of gene rearrangement
which NOD may be important in Crohn’s diseasde
NOD2
chediak higashi gene and highlights
CHS1 mutation
defect in vesicular transport proteins needed for formation or exocytosis of melanosomes
sxs: partial loss of skin/hair pigmentation, HLH, inability to clear EBV
are lesions borders in contact dermatitis poorly or well defined?
poorly defined because there can be secondary spread
most frequent cause of occupational asthma in western countries
baking products
what agent is used extensively in spray paints
hexamethylene diisocyanate
*carries better prognosis than other diisocyanates
how do corticosteroids affect neutrophils and eos
inhibit apoptosis of neutrophils
promote apoptosis of eosinophils
chemoattractant that attracts eosinophils to the airway mucosa in asthma?
CXCL5
Which cytokines are produced most abundantly by CD4+ T lymphocytes in allergic asthmatic airway inflammation?
GM-CSF
IL4, 5, 9, 13
which virus is ass’d with asthma exacerbations
rhinovirus C
how long does cough need to be in children to be chronic
> 4 weeks
most common cause of upper airway cough syndrome
bacterial sinusitis
most common causes of chronic nonspecific cough in children (2)
asthma
protracted bacterial bronchitis
how long does chronic cough from GERD take to improve after tx is started
up to 3 months for improvement (6 months to resolve)
dominant allergen in egg white
ovomucoid (Gal d1)
What percentage of children with milk allergy typically tolerate extensively heated milk in baked products?
75%
heiner syndrome
food induced pulmonary hemosiderosis
recurrent PNA ass’d with pulm infiltrates and hemorrhea, hemosiderosis, GI blood loss, IDA, and FTT
most often ass’d with non-IgE mediated hypersensitivity to cow’s milk
IL-33 may be important in anaphylaxis due to:
its direct induction of IgE sensitized mast cell degranulation and cytokine production
main pathophysiologic feature of anaphylactic shock is:
fluid extravasation causing hypovolemia and hemoconcentration
The reason that initial treatment for anaphylactic shock is unsuccessful is most often due to which of the following?
insufficient fluid resuscitation
which medication is ass’d with enhanced susceptibility to episodes of scombroidosis?
isoniazid
During an additive challenge for asthma, what percent decrease from baseline FEV1 is considered a positive response?
> 20%
Which sulfite-containing medication is considered safe for patients with sulfite-induced asthma?
epinephrine
what dz has MSG been ass’d with
persistent rhinitis
In what gene does polymorphism modify the effect of farm environmental endotoxin exposure on allergic sensitization in children?
CD14
what eye conditions can typical cyclosporine be used for
dry eye syndrome
AKC
VKC
what allergy med interacts with theophylline
zileuton can cause increase in theophylline
what characteristics in asthmatic children predicts ICS response (2)
AEC >300
aeroallergen sensitization
what treatment does GLILD seem to respond to
systemic steroids
diagnostic criteria of ABPS (3)
1) asthma or CF
2) sIgE to A fumigatus
3) total IgE >1000
which meds blunt ASA reactions in AERD (3)
zileuton
xolair
montelukast
*mepo does not although it’s approved to treat asthma and NP in pts with AERD
drug inducing most reactions
PCN
most common intraop anaphylaxis agent in US
antibiotics
MOA of benralizumab
targets alpha subunit of IL5R –> eos apoptosis by ADCC
Fc portion binds FcyRIII on NK cells
which biologics for asthma haven’t shown a decrease in PO steroid in steroid dependent asthma
tezepelumab
xolair
treatment of EGPA ass’d myocarditis
steroids + cyclophosphamide
features of hypocomplementemic urticarial vasculitis syndrome
> 6months urticaria and low C1q +/- low C3/C4
angioedema, COPD, uveitisi/episcelritis, arthritis, glomerulonephritis
DOCK8 and STAT3 LOF Job syndromes differentiators
DOCK8 - severe warts
Jobs - scoliosis, pneumatoceles
cause of autosominal dominant IFNgR1 deficiency
mutation that removes signaling and recycling domains of IFNgR1 -> remnant protein dominantly interferes with signaling and accumulates on cell surface
5 major organisms causing infections in CGD patients in N America
s aureus
serratia marsescens
burkholdia cepacia
nocardia
aspergillus
hallmark NK cell deficiency infection
herpesvirus
what innate signaling mechanism promote a Th17 response in candida infections
CLR signaling (innate immune receptor)
chance of siblings being an HLA match
25%
ILC3 are mimics of what T helper subset
Th17 - both depend on transcription factors RORgt and produce IL17 and IL22
feature of eos activation
increased lipid bodies containing eicosanoid products
what is TACE
TNF alpha converting enzyme aka ADAM 17 endopeptidase
secreted by TNF producing cells, cleaves membrane extension anchoring TNF to release it form the cell
[ ] of free serum IgE in healthy humans
50-200 ng/mL (1-10 for other IGs)
units for non standardized allergen extracts
PNU/mL or w/v
what intervention is most strongly ass’d with decreased resp disease from air pollution
public policy measures
