Module 6

Question 1

Some mechanisms that contribute to immunological self-tolerance

Answer 1

negative selection of B cells and T cells, exclusion of lymphocytes form some peripheral tissues (brain, eyes, testes), anergy in autoreactive cells that make it to the periphery, suppression of autoimmune responses by regulatory T cells

Question 2

hypersensitivity reaction developed against self-antigens

Answer 2

autoimmunity

Question 3

3 factors that can contribute to autoimmunity

Answer 3

infections (esp viral), molecular mimicry from pathogens, and tissue damage/injury releasing self-antigens

Question 4

when some pathogens that express antigens that resemble self-antigens

Answer 4

molecular mimicry

Question 5

disease caused by Strep pyogenes that creates autoantibodies that cause myocarditis, nephritis, and arthritis

Answer 5

Rheumatic fever

Question 6

Some non-infectious triggers of autoimmunity

Answer 6

toxins, drugs, hormones, loss of regulatory cells

Question 7

these places are separate from circulation and have mechanisms to inhibit self-antigenic response either through regulatory or anatomical barriers

Answer 7

immunologically privileged sites

Question 8

Examples of immune privileged sites

Answer 8

brain, eyes, testes, and uterus

Question 9

damage to the eye that initiates autoimmunity and destroys vision

Answer 9

sympathetic opthalmia

Question 10

Examples of type II autoimmune reactions

Answer 10

hemolytic anemia, myasthenia gravis, graves disease

Question 11

Example of cytotoxic/cytolytic reaction

Answer 11

autoimmune hemolytic anemia

Question 12

What antibodies are generated against RBC antigens in hemolytic anemia?

Answer 12

IgG and IgM

Question 13

Non-cytotoxic/cytolytic reactions

Answer 13

Myasthenia gravis and Grave’s disease

Question 14

Does inflammation occur in non-cytotoxic reactions?

Answer 14

no

Question 15

Disease where antibodies are generated against the Ach receptor and inhibit binding of Ach to AchR

Answer 15

myasthenia gravis

Question 16

Most common cause of hyperthyroidism

Answer 16

Grave’s disease

Question 17

Disease where antibodies are generated against the TSH receptor and leads to hyperthyroidism, goiter, exophthalmos, tremors, tachycardia, diaphoresis

Answer 17

Grave’s disease

Question 18

Examples of Type III autoimmune reactions

Answer 18

SLE and rheumatoid arthritis

Question 19

immune complex diseases are also what type of autoimmune reaction?

Answer 19

Type III

Question 20

What reaction occurs when immune complexes get trapped in the basement membrane of small blood vessels, cause complement activation and degranulation?

Answer 20

Type III/immune complex diseases

Question 21

Where do immune complexes accumulate in SLE?

Answer 21

kidneys, joints, and blood vessels (esp in the cheeks to give butterfly rash)

Question 22

self-antigens often found in SLE

Answer 22

nuclear antigens

Question 23

Rheumatoid factors are usually which antibodies?

Answer 23

IgM that reacts with the Fc of IgG usually and creats an IgM-IgG immune complex

Question 24

Which disorder can be a type III or type IV reaction?

Answer 24

Rheumatoid arthritis

Question 25

What does IgM and/or IgG form against in RA?

Answer 25

synovial joint antigens

Question 26

How can RA lead to a type IV reaction?

Answer 26

by fixing complement

Question 27

Examples of Type IV autoimmune reactions

Answer 27

RA, insulin dependent DM, MS

Question 28

What's the difference between a type III and type IV RA reaction?

Answer 28

type III: immune complexes deposit in the joints. Type IV: autoreactive CD4 T cells activate macrophages which release cytokines and can cause systemic inflammation

Question 29

Cytokine involved in RA

Answer 29

TNF-alpha

Question 30

What type of DM is insulin-dependent DM?

Answer 30

Type 1

Question 31

insulin producing cells in pancreas

Answer 31

beta islet cells

Question 32

What type of T cells attack the beta islet cells in T1DM?

Answer 32

CD8 T cells

Question 33

Disease characterized by an autoimmune t cell response against myelin

Answer 33

MS

Question 34

What type of T cells activate macrophages to release proteases and cytokines in MS?

Answer 34

CD4 T cells

Question 35

genetic immune deficiencies are msot often caused by what kind of gene defects and on what chromosome?

Answer 35

recessive and on X chromosome

Question 36

When are most primary immune deficiencies diagnosed?

Answer 36

after 6 months of age after maternal/placental IgG has waned

Question 37

What defects can cause inherited primary immune deficiencies?

Answer 37

defects in phagocytes, antibody production or isotype switching, B cells, T cells, complement factors, cytokine production or receptors, or MHC II or II deficiency

Question 38

How do secondary immunodeficiencies develop?

Answer 38

they are acquired

Question 39

recurrent bacterial and fungal infections often signal what?

Answer 39

phagocyte deficiencies

Question 40

Examples of phagocyte deficiency disorders

Answer 40

chronic granulomatous disease and myeloperoxidase deficiency

Question 41

disease where neutrophils cannot produce superoxide radicals and bactericidal activity is reduced

Answer 41

Chronic granulomatous disease

Question 42

NADPH oxidase defect is associated with what disease?

Answer 42

chronic granulomatous disease

Question 43

Disease where myeloperoxidase affects the ability to convert hydrogen peroxide to hypochlorite

Answer 43

Myeloperoxidase deficiency

Question 44

A deficiency in this system leads to impaired humoral immune function and persistence of immune complexes

Answer 44

complement deficiency

Question 45

Mutations in which complement pathway make a patient more susceptible to extracellular pathogens due to accumulation of opsonized bacteria?

Answer 45

classical pathway

Question 46

Improper formation of what leads to impaired defense against Neisseria?

Answer 46

membrane attack complex

Question 47

What are some examples of antibody deficiency disorders?

Answer 47

Bruton X-linked agammaglobulinemia (XLA), X linked hyper-IgM syndrome, Selective IgA deficiency

Question 48

Disorder resulting from a loss of Btk and causes a loss of B cells

Answer 48

Bruton's X-linked agammaglobulinemia (XLA) (think BBB: bruton, Btk, B cell)

Question 49

Protein kinase involved with XLA

Answer 49

Bruton's tyrosine kinase (btk)

Question 50

Which stage of B cell maturation is affected by XLA?

Answer 50

pre-B cell stage

Question 51

CD40L is altered in what disorder?

Answer 51

X linked hyper-IgM syndrome

Question 52

What disorder occurs when T cells cannot activate B cells via CD40/CD40L?

Answer 52

X linked hyper-IgM syndrome

Question 53

Most common immune deficiency

Answer 53

selective IgA deficiency

Question 54

What are some examples of lymphocyte deficiencies?

Answer 54

Severe combined immune disease (SCID), Bare lymphocyte syndrome Iand II (BLS I and II), DiGeorge syndrome

Question 55

What disorder is characterized by complete deficiency in T and B cells, often caused by a mutation of RAG-1/2?

Answer 55

Severe combined immune disease

Question 56

What disorder is classified by a deficiency of MHC class I and CD8+ T cell?

Answer 56

Bare lymphocyte syndrome I (BLS I)

Question 57

What disorder is classified by a deficiency of MHC class II and CD4+ cells?

Answer 57

Bare lymphocyte syndrome II (BLS II)

Question 58

What disorder is characterized by abnormal thymic epithelium and a failure of T cell development?

Answer 58

DiGeorge syndrome (22q11.2 deletion syndrome)

Question 59

What is the usual treatment for immunodeficiencies?

Answer 59

IVIG