Immunopathologies/Immunology Flashcards Preview

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Flashcards in Immunopathologies/Immunology Deck (163):
1

Contact Dermatitis

Type IV, reaction to topic chemicals like henna, latex, nickel, hair dyes, tattoos

2

Rheumatic Heart Disease - Symptoms

Heart Murmur

3

Infections associated with T-cell Defieincy

Candida Albicans, Pneumocytsis Jirovecii (bactieral, virus, yeast, fungi)

4

Dressler's Syndrome Treatment

usually self limited, stops when heart heals; or with autoimflammatory

5

CVID - mechanism

Normal Pre-B and B-cells, but B-cells are unable to make antibody intermittently; group of ~20 conditions

6

second graft reaction

45-10 days due to memory cells

7

Chronic Beryllium disease Mixed

4: T cells damage (HLA-DP1); CFR

8

Rheymatoid Factor

Type 3, pts serum to IgG coated microbeats. IgM anti-IgG will agglutinate.

9

Arthus Reactions

Type 3: if preexisitng ab (during booster) will form complex at injection site and causes fussiness and sore arm

10

What did David - Bubble Boy die from?

EBV infection - latent in everyone except we have functioning T-cells

11

CVID - name

Common Variable Immunodeficiency

12

Diagnosis of Asthma

Spirometry - measure air flow. Forcibly exhaled form full lungs (1 sec) + same with broncodilater. If improvement = asthma

13

Release of sequestered antigen

method in Type II immunopathy; immune response is generated to release antigen from store

14

Mutation in Celicas

90% are HL-DQ2

15

Prevalence of SCID

50 in 100,000

16

Type II immunopathy - basic

IgG, IgM, IgA autoantibodies to specific Host Tissue

17

Viral illnesses with secondary immunodeficiency

mononucleosis, cytomegalovirus, AIDs

18

Why does a TB test work?

Intradermal injection of antigen (PPD) that measure Th1 activation. (Type IV)

19

DiGeorge Syndrome Mechanism

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

Prevalence of Bruton’s

0.4 in 100,000

21

Myasthenia Gravis - Treatment

Thymectomy, immunosupresssion, nestigmine - drug to increase ACh effectiveness

22

Rheumatic Heart Disease Pathophys

Type II; Cross reaction between Group A Strep M protein to Laminin protein on heart valves, followed by neutrophil mediated destruction

23

Hyperacute graft reaction

Rejection before healing, bloodless graft, common in human-animal transplants due to predisposing antibody

24

Paroxysal Cold Hemobloginuria

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.