Immunopathologies/Immunology Flashcards
1
Q
Contact Dermatitis
A
Type IV, reaction to topic chemicals like henna, latex, nickel, hair dyes, tattoos
2
Q
Rheumatic Heart Disease - Symptoms
A
Heart Murmur
3
Q
Infections associated with T-cell Defieincy
A
Candida Albicans, Pneumocytsis Jirovecii (bactieral, virus, yeast, fungi)
4
Q
Dressler’s Syndrome Treatment
A
usually self limited, stops when heart heals; or with autoimflammatory
5
Q
CVID - mechanism
A
Normal Pre-B and B-cells, but B-cells are unable to make antibody intermittently; group of ~20 conditions
6
Q
second graft reaction
A
45-10 days due to memory cells
7
Q
Chronic Beryllium disease Mixed
A
4: T cells damage (HLA-DP1); CFR
8
Q
Rheymatoid Factor
A
Type 3, pts serum to IgG coated microbeats. IgM anti-IgG will agglutinate.
9
Q
Arthus Reactions
A
Type 3: if preexisitng ab (during booster) will form complex at injection site and causes fussiness and sore arm
10
Q
What did David - Bubble Boy die from?
A
EBV infection - latent in everyone except we have functioning T-cells
11
Q
CVID - name
A
Common Variable Immunodeficiency
12
Q
Diagnosis of Asthma
A
Spirometry - measure air flow. Forcibly exhaled form full lungs (1 sec) + same with broncodilater. If improvement = asthma
13
Q
Release of sequestered antigen
A
method in Type II immunopathy; immune response is generated to release antigen from store
14
Q
Mutation in Celicas
A
90% are HL-DQ2
15
Q
Prevalence of SCID
A
50 in 100,000
16
Q
Type II immunopathy - basic
A
IgG, IgM, IgA autoantibodies to specific Host Tissue
17
Q
Viral illnesses with secondary immunodeficiency
A
mononucleosis, cytomegalovirus, AIDs
18
Q
Why does a TB test work?
A
Intradermal injection of antigen (PPD) that measure Th1 activation. (Type IV)
19
Q
DiGeorge Syndrome Mechanism
A
Large 45 deletion on Ch22 to cause absence of parathyroid gland due to ineffective stroma of pharyngeal cleft. Most due to repeated mutations or de-novo. Absent T-cells, normal B-cells
20
Q
Prevalence of Bruton’s
A
0.4 in 100,000
21
Q
Myasthenia Gravis - Treatment
A
Thymectomy, immunosupresssion, nestigmine - drug to increase ACh effectiveness
22
Q
Rheumatic Heart Disease Pathophys
A
Type II; Cross reaction between Group A Strep M protein to Laminin protein on heart valves, followed by neutrophil mediated destruction
23
Q
Hyperacute graft reaction
A
Rejection before healing, bloodless graft, common in human-animal transplants due to predisposing antibody
24
Q
Paroxysal Cold Hemobloginuria
A
a type of AIHA that causes hemolysis after exposure to cold.
25
Rheumatoid arthritis
Type 3,, trapped IgM to IgG complexes; elemetns of type 2 and 4
26
Autosomal Recessive SCID
Adenosine Daminase Deficiency ADA
27
Prevalence of DiGeorge
30 in 100,000
28
BCG vaccine
Vaccine against TB
29
Autoimmunity to Interferon Gamma
appears to be immunodeficient; often diagnosed due to rapid onset of TB in previously healthy patient
30
secondary immunodeficiency
immunodeficiency due to immunosuppressant drugs, advanced cancer, measles, AIDS
31
Quantifero
uses PPD from human mixed with blood sample and measures IFN-gamma. Only active TB (from actual exposure) will elicit a reaction
32
Autoimmunity Thrombocytopenic Purpura - Pathophys
Platelets are opsonized by AutoAb for destruction in spleen, Type II
33
Hybrid
method in Type II immunopathy; foreign antigen couples to self so anti-self B-cell ingest and normal Tfh is activated by foreign antigen to help class switch
34
Poison Ivey
urushiol oil causes delayed hypersensitivity reacion (Type IV)
35
Type III choroid plexus
confsion/dementia
36
Sjoren’s Syndrome
Type IV, unkown mechanism that causes CTL action against exocrine glans and dry saliva and tears
37
Symtoms of CVID
problems with innate immunity; recurrent bacterial infections, increased risk of lymphoma, enteropathy, autoimmunity, mild immunodeficiency compared to others
38
Mutations in SCID
SCID-X1; ADA; V(D)J recombination defects
39
Celiacs
Ab to gut endomyosium. Transglutaminase 2 (TG2) crosslinks normally with glutin, but in celiac it crosslinks with what gladden (digestion resistant) and becomes an antigen via an illicit help mechanism (T-cell mediated inflammation to gliadin) cause villi blunting
40
Nucala
reduces asthma by reducing blood eosinopohils
41
Histamine
itch, blood vessel dialation, leakiness
42
Renal biopsy in type 3
lumpy and bumpy with fluorsecence.
43
Symptoms of Bruton’s Agammaglobinemia
bacterial infections (less susceptible to viral infections), pneumonia, chronic diarrhea, enterovirus infections (due to no IgA) —\> poliovirus
44
Chronic Frustrated Response
Noramlly TGFbeta+IL10 promote Treg, but dysregulation and release of TGFBeta and IL6 decrease Treg and increase TH1,2, 17 leading to chronic inflammation.
45
Basic Mechanism of SCID
Low T and B cell due to block in development of Lymphoid System; most severe
46
Ataxia Telangiectasia mechanism
Both T and B cell deficiency and severe IgA depression due to defect in DNA repair
47
Diagnosis of Type 3
History, CH50, Immune complexes in blood (cryoglubulins), Rhematoid factor, Renal biopsy,
48
Juvenile diabetes mixed
Type 2: ab to islet 4: T cells damage by HLA-DR3 and DR4
49
Oral Allergy Syndrome
Type I; food/antigen penetrates MM in mouth to activate mast calls, tingling lips and toungue, swelling, itching. From foods altho are similar to pollens
50
Type III immunopathology
Ab-Antigen complexes of intermediate size get stuck in basement membrane and activate complement (C1q); C3a and C5a atttrached neutrophils to release inflammatory mediators
51
Immunotherapy mechanism
favors IgG rpoduction so IgG traps and clears allergen before loaded on IgE mast cells.
52
Treatment of ADA
Irradiated blood (RBC are risk of adenosine deaminase to kill lymphocytes) or purified ADA Stabilized with polyethylene glycol
53
Hashimoto’s thyroiditis mixed
2: ab to thyroid; 4; tyroid infiltrated with T-cells to cause damage. Hypothyroidism
54
HyperIgM Mechanism
CD40 (on B cell) or CD40L (ligand on Tcell) is defective, so Tfh can’t recognize B-cell to help class switch.
55
Treatment of B-Cell Defienct Disorders
Human Ig - IVIG and SCIG given monthly
56
Autoimmune encephalitis
using brain cells as antigen and presenting to immune cycle to activate T-cells so they are more primed to enter BBB
57
Memory T-cell
decrease threshold of antigen exposure to elicit immune reponse
58
SLE
Type 3, IgG to dsDNA (own DNA) that deposits in the kidney (glomerulonephritis)
59
Type III pleura
pleusiy, effusion
60
infections associated with B-Cell Deficiency
Extracellular, pyrogenic - pus producing bacteria - staph, H influenza, Strep
61
Type I Immunopathology
allergic reaction that induces IgE, IgE beings to FceR1on mast cells that are cross linked to allergens to induce release of histamine.
62
Mutation in Bruton
Bruton Tyrosine Kinase (btk) defect, a tyrosine Kinase expressed in pre and mature B-cells
63
Chronic Spontaneous Uritcaria
IgG antibody to FceR1 on mast cells to chronically release histamine
64
Allergic seasonal rhinitis
hay fever, funny nose, itchy eyes in response to pollen, cats/dogs. Type I
65
Innocent Bystander
method in Type II immunopathy; damage to normal tissue that is associated or infected with antigen (drug that binds to RBC, ab to drug)
66
Diagnosis of Type I
hisotry, skin testing, RAST (ardio-allergo sorbent tesst) of ImmunoCAP-FEIA (samples serum combined with allergen fixed to capsule and added to serum. Unbound proteins washed away, and bound IgE revealed - safe method)
67
Most SCID happen due to this mutation…
SCID-X1, an X linked Recessive defect in Gamma Chain that forms receptors IL-2
68
Treatment of Immunodefiencies
Isolation; Prophylactic antibodies (combination, change monthly); Human Ig, Transplant
69
Laboratory findings with Bruton’s
Pre-B cells in marrow; but deficiency in Bcells and antibody in periphery (Ig
70
Mechanisms of Type II immunopathy
1) neutralization: human protein inactivated by autoAb 2)Complement: auto activates complement to induce lysis, phagocytosis, lysosomal enzymes 3) Stimulatory Hypersensitivity: autoAb directed against cell surface receptors and behaves like agonist
71
One shot serum sickness
type III one shot of rabbit or horse serum to treat ID and 10-14 days later fever, malaise, rash, itch, arthralgia, hives, lymphadenopathy.; can be due to drug coupling with host proteins and viral infections
72
Para-Phenylaminediame
black henna that elicits a DTH
73
Signs/Symptoms of Digeorge
CATCH-22” Calcium (convlusions- inability to regulate calcium), Appearance (wide eyes, low set ears, fish mouth), Thymus (immunological disease), Cleft (palate), Heart (heart vessel abnormalities, tetralogy of flow, ASD, VSD)
74
Multiple Sclerosis - pathophys
Type IV; T-cells reactive to Myelin Basic Protein. Brain is antigenic but NOT immunogenic
75
Dressler’s Syndrome - Symptoms
Persistent cardiac pain, fever, malaise, pericardial effusion after heart attack or heart surgery
76
Periodontal disease treatment
immunsuppresion, someontes ab to IL6 receptor
77
Graves’ Disease
Stimulatory Autoimmunity, IgG resembles TSH to stimulate thyroid and cause hyperthyroidism; Type II
78
Type III kidney
glomerulonephritis
79
Bruton’s Agammaglobinemia
X linked; normal T-cells, but low to absence B- cells. Block between Pre-B and Immature B-cell(with IgD or IgM on surface)
80
Dermatitis herpetiformis
skin manifestation of celiacs, IGA to TG3 in skin
81
Autoimmunity Thrombocytopenic Purpura - symptoms
Bleeding abnormalities in young, healthy post viral infection or Older patient with autoimmunities; or from certain drugs
82
HyperIgM Syndrome
Normal IgM, but can’t class switch to IgG or IgA (low levels); X-linked
83
will the bump be hard or soft for a positive PPD test?
Hard - full of macrophages (type I) due to Th1 recruiting thousands of macrophages)
84
Chron’s
CFR: large and small intestine, esp terminal illeum, patchy microabcesses that become granulomas
85
Ataxia Telangiectasia signs and symptoms
AR; sinus infections, pneumonia, ataxia, telangiectasia (dilated abnormal vessles), tumors
86
Rescue inhalers
short acting Beta2 antagonists - albuterol
87
Type III in joints
athritis
88
IgA Nephropathy
Type 3,asymptomatic, but presents with hematuria. IgA nephropathy causes terminal sugars in hinge to go missing and this causes cross reaction with normal IgA and IgG in renal glomerulus.
89
Type III Peritoneum
Sterile peritonitis
90
Why don’t we use oral polio virus vaccine anymore?
because people who have Bruton Agammaglobulemia are more susceptible to poliovirus (and all enterovirus) that is associated with the live virus.
91
Common characteristics of all immunodeficiency diseases
Diarrhea and malabsorption of nutrients
92
T-Cell Testing
Skin test to recall Ag panel, Total lyphocyte count, CD3, CD4, CD\* THEN mitogen response, mixed leukocyte reaction, cytokine measurements, sequence suspect gene
93
Adenosine Deaminase Deficiency (ADA)
adenosine accumulate in cells to impair lymphocyte development; AR form of SCID
94
Psoriasis
CFR, Th17 dominated, associate with HLA-Cw\*06:02
95
Autoimmunity Hemolytic Anemia
Type II; Ab against RBCs usually post viral infcetion, with other autoimmune, cancer, or drugs
96
primary immunodeficiency
mutations in gene required for normal development of immune system
97
Chronic Beryllium Disease
(Type IV) Reaction of Be to cause scarring of lung tissue; related to HLA-DP1
98
Post-Strep Glumerulonephritis
Type 3, 10-14 days post infection with strep, scarlet fever, impetigo of skin - NV, fever, malaise, hypertension, reduced urine output, hmaturia, joint paint and rash. disease is self limiting
99
X-linked SCID
SCID-X1
100
Type I diabetes
Type IV, autoab attack to beta islet clls in pancreas; HLA DR3 and DR4 are in linkage disequilibrium with DQ2 and DQ8 so they more readily bind to islet cells and present as foreign
101
PPD on tests with BCG vaccine?
will also be Positive; must instead use Quantifiro test
102
IgE production
Tfh/IL4 dependent. Slow process and requires multiple exposures.
103
What was David - Bubble Boy’s treatment?
mismatched sisters BM with anti CD4 and CD8 and compliant to rid of mature T-cells to eliminated graft vs. host reaction
104
Treatment of DiGeorge
Fetal thymus or thymus stromal transplant (at least 1 class I and Class II)
105
Cross-Reaction
method in Type II immunopathy; between foreign antigen and self antigen
106
Mechanisms of loss of tolerance in Type II immunopathy
Hybrid; Forbidden clone; Cross reaction; Passive ab; innocent bystander; release of sequestered antigen; failture of regulatory mechanism
107
First Graft Rejection
10-20 days
108
GoodPasture’s Syndrome - Symptoms
Glomerulonephritis, pneumotis, pulmonary hemorrhage
109
Forbidden clone
method in Type II immunopathy; autoreactive T-cell escape
110
Myasthenia Gravis - Mechanism
Type II immunopathy, Aire, a TF, doesn’t promote the synthesis of CHRNA1, which codes for alpha AchR, so AChr is not expresse din thymus and negative selection does not occur. T-cells then activate B-cells to release Ab against AChR in NMJ
111
Complement Testing
CH50, assay for CH1inhibitor, THEN individual complement component levels
112
Dressler's Syndrome - Pathophys
Type II; AutoAb to pericardial of myocardial antigens
113
Two phase of type 1 immunopathology
Early: histamine and can be treated with antihistamine, late: 4-10 hours laters, Prostagalingin, Luekotriene and cytokine release in inflammaotry. Treated with antiinflammaotry
114
Chronic Beryllium Disease
CFR: inhaled Be covalently links to peptides to create neoepitope that riggers Th1 and later Th2. Cant be removed by macrophages. Chronic after 1 exposure. Linked to HLA-DP allel to create pocket for Be+
115
Rheumatic Fever
different from Rheumatic heart disease in that it involves the Skin and CNS
116
Lab values of CVID
Serum IgG
117
Treatment of Type I immunopathology
avoid allergn, antihistamine, epineprine, steroids, leukotriene inhibit, long acting beta-2 antagonists, IgE blockers, immunotherapy
118
Myasthenia Gravis - Symptoms
Progressive Muscle Weakness
119
Late phase IgE reaction
PLA2 cleaves AA to make prostaglandin and luekotrienes, (4-10 hours later).
120
Immediate IgE reaction
IgE binds to mast cells and basophils with IgE receptor FCeR1. high binding affinity. IgE relelases granules when two adjacent IgE molecules are cross linked by allergen.
121
Selective IgA Deficiency
Most common immunodeficiency 1/500; mostly asymptomatic, bu can cause diarrhea, sino-pulmonary infection, increased severity of allergies; 10-15X more common in celiac patients
122
Celicas Mixed
2: ab to tissue transglutaminase; CFR
123
Biopsy of Type I Infections
infiltration of Th2 and eosinophils
124
LABAs
long acting beta 2 agnosts - reduce bronchoconstriction for 12 hours - advair
125
IBD
CFR: Genetic component with NOD2 (A PRR), but alos due to increase permeatbility of gut so secreted defense resturns to gut to activate DAMPs to increase IL6 and more TH1,2,17 against gut flora.
126
Secondary immunodeficiency Drugs
Corticosteroids, monoclonal antibodies
127
B-Cell Testing
Serum protein electrophoresis, quantitative IgG, IgA, IgM, sepcific Abs prior to immunization, ABO isohemagglutinins BEFORE Ab response to noveal Ag, sequence, lymph node biopsy
128
Type IV Immunopathy - basics
Delayed Hypersensitivity reaction; T-cell mediated - does not require Ab
129
SLE mixed
2: AutAb to dsDNA; 3: IgGdsDNA complex in kidney; 4: T cell mediated damage
130
Conditions of Graft vs. Host
Graft must have T-cells, by antigenic, and be in an immunocompromised host
131
Mixed RA
TYpe II: IgM against own IgG(Rheumatoid factor); Type III: trapped IgM and IgG complex; Type IV: T\_cell mediated damage
132
Passive Antibody
method in Type II immunopathy; hemolytic disease in newborn, mismatched transfusion, child of mother who has myasthenia gravis or SLE
133
Lab findings of SCID
Lymphopenia in Both B and T cells; thymus shadow on X-ray, small tonsils, low mitogen response, low serum Ig
134
UC
CFR: more superficial, in large intestine, leads to bleeding
135
Wiscott-Aldrich
X linked; platelet and B-cell cell deficiency, eczema and bacterial infection
136
HLA-B\*5071
Abacavir hypersensitivity; in linkage disequilibirum with actual causal HLA-D gene. a Class I gene, so it elicits a CTL problem
137
Goodpasture's Syndrom mechanism
Type II immunopathy; AutoAb to Type IV collagen found in lung and kidney of Basement Membrane. Appears linear, not lumphy and bumpy with immunofluorescence
138
what inflammatory mediators are involved in Type III
H2O2, proteinases, histamine
139
Periodontal Disease
CFR major tooth loss, bacterial live in giginval crevice where T-cells can’t reach. Shift to increase IL-6/TGFbeta and we see Ab to citrullinated proteins lik in RA.
140
Treatment of SCID
BMT (50% success rate); better to use purified hematopoetic stem cells
141
Graft vs. Host
Type IV; acute (2-10 weeks; maculopapular rash, diarrhea, hepatic inflammation, infection) or Chornic (months to years) or vs. Leukemia.
142
LIPT
sublingual immunotherapy for ragweed allergies
143
Asthma
Type I, bronchoconstrictive iflammaotry, need to treat to avoid lung fibrosis
144
Diagnosis of Type II immunopathologies
Direct or Indirect Immunofluorescence
145
V(D)J
defect in SCID that is most common in navajo and apache children
146
Treatment of Type I immunopathology
Avoidance, Antihistamines, epinephrine, steroids,Luekotriene inhibitors, rescue inhalers, long acting beta2 agonists, IgE blockers, Immunotherapy
147
Eczema
Atopic derm, chronic dry and irritated skin, rash, self worsening (itching release more inflammatory cytokines), secondary infect common Type I; cross reactions are common
148
Hypersensitivity Penumonitis
Type 3 Farmers lung, exposure to filamtenous bacteria in modly hay that causes serum IgG. when inhaled enough, forms complex and auses shortness of breath, dry gcough, malaisem tachycardia. if progessive, goes to type IV. treatment with steroids and avoidance
149
Th2
helps switch B Cells ot make IgE. Release IL4, IL5, IL13 to activate M2 macrophages to heal damage and wall off.
150
what is important to look for physical exam for immunodefiencies?
Development, tonsils, lymph nodes
151
SCID - name
Severe Combined Immunodeficiency Disease
152
Nude Mice
different mutation, but also fail to develop thymic stroma and have same characteristics of Digeorge
153
Phagocyte Testing
WBC count, differential, morphology, NBT test, oxidative burst THEN assay for phagocytosis, chemotaxis, sequence
154
Hyper IgE Syndrome
AD, inability to make IFN gamma (jobs Syndrome) which leads to Th2 dominance over Th. High serum IgE leads to skin abcesses, fungal or pseudomonas pneumonia
155
Paraiste resonse
Type 1: IgG binds to worm or ova and activates complement, C3a and C5a attract neutrophils but nothing happens. IgE diffuses to nearby Mast cells and release histamine for smooth muscle contraction and peristalsis. Eosinophils are activated by IgG to release granules of Major Basic Proteins to kill worm.
156
Antigens in type III
must be present in sufficient amount to form complexes (or depot of antigen)
157
Steps in Type IV Immunopathy
Initiation: APC presents to Tcells, but not fast enough to generate reaction; Elicitation: T-cell response with peak of 24-48 hours due to memory T-cells
158
Transient Hypogammaglobulinemia of Infancy
from 3-6 months up until 18th month; IgG production is low and results in recurrent and persistent gram positive infections, 15% of infant diarrhea
159
Atopic state
predisposition to allergies
160
Abacavir reaction
(Type IV) reaction that causes rash, malaise, variable GI, Respiratory tract, MSK reactions. Due to HLA-B\*5071
161
Lawsonine
good type of henna with no DTH
162
Failure of Regulatory Mechanisms
method in Type II immunopathy; imbalance in type of T-cells? still undecided whether pathologic or therapeutic
163
CH50
Type 3, total hemolytic complement levles. Pt serum is diluated and added to antibody coateded RBCs, maximal dilution to causes 50% of cells to lyse compared to standard.
