Module 6 Flashcards

Autoimmunity

You may prefer our related Brainscape-certified flashcards:
1
Q

Some mechanisms that contribute to immunological self-tolerance

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

hypersensitivity reaction developed against self-antigens

A

autoimmunity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

3 factors that can contribute to autoimmunity

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

when some pathogens that express antigens that resemble self-antigens

A

molecular mimicry

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

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

A

Rheumatic fever

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Some non-infectious triggers of autoimmunity

A

toxins, drugs, hormones, loss of regulatory cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

immunologically privileged sites

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Examples of immune privileged sites

A

brain, eyes, testes, and uterus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

damage to the eye that initiates autoimmunity and destroys vision

A

sympathetic opthalmia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Examples of type II autoimmune reactions

A

hemolytic anemia, myasthenia gravis, graves disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Example of cytotoxic/cytolytic reaction

A

autoimmune hemolytic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What antibodies are generated against RBC antigens in hemolytic anemia?

A

IgG and IgM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Non-cytotoxic/cytolytic reactions

A

Myasthenia gravis and Grave’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Does inflammation occur in non-cytotoxic reactions?

A

no

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

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

A

myasthenia gravis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Most common cause of hyperthyroidism

A

Grave’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

Grave’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Examples of Type III autoimmune reactions

A

SLE and rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

immune complex diseases are also what type of autoimmune reaction?

A

Type III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

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

A

Type III/immune complex diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Where do immune complexes accumulate in SLE?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

self-antigens often found in SLE

A

nuclear antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Rheumatoid factors are usually which antibodies?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

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

A

Rheumatoid arthritis

25
Q

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

A

synovial joint antigens

26
Q

How can RA lead to a type IV reaction?

A

by fixing complement

27
Q

Examples of Type IV autoimmune reactions

A

RA, insulin dependent DM, MS

28
Q

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

A

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

29
Q

Cytokine involved in RA

A

TNF-alpha

30
Q

What type of DM is insulin-dependent DM?

A

Type 1

31
Q

insulin producing cells in pancreas

A

beta islet cells

32
Q

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

A

CD8 T cells

33
Q

Disease characterized by an autoimmune t cell response against myelin

A

MS

34
Q

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

A

CD4 T cells

35
Q

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

A

recessive and on X chromosome

36
Q

When are most primary immune deficiencies diagnosed?

A

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

37
Q

What defects can cause inherited primary immune deficiencies?

A

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

38
Q

How do secondary immunodeficiencies develop?

A

they are acquired

39
Q

recurrent bacterial and fungal infections often signal what?

A

phagocyte deficiencies

40
Q

Examples of phagocyte deficiency disorders

A

chronic granulomatous disease and myeloperoxidase deficiency

41
Q

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

A

Chronic granulomatous disease

42
Q

NADPH oxidase defect is associated with what disease?

A

chronic granulomatous disease

43
Q

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

A

Myeloperoxidase deficiency

44
Q

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

A

complement deficiency

45
Q

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

A

classical pathway

46
Q

Improper formation of what leads to impaired defense against Neisseria?

A

membrane attack complex

47
Q

What are some examples of antibody deficiency disorders?

A

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

48
Q

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

A

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

49
Q

Protein kinase involved with XLA

A

Bruton’s tyrosine kinase (btk)

50
Q

Which stage of B cell maturation is affected by XLA?

A

pre-B cell stage

51
Q

CD40L is altered in what disorder?

A

X linked hyper-IgM syndrome

52
Q

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

A

X linked hyper-IgM syndrome

53
Q

Most common immune deficiency

A

selective IgA deficiency

54
Q

What are some examples of lymphocyte deficiencies?

A

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

55
Q

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

A

Severe combined immune disease

56
Q

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

A

Bare lymphocyte syndrome I (BLS I)

57
Q

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

A

Bare lymphocyte syndrome II (BLS II)

58
Q

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

A

DiGeorge syndrome (22q11.2 deletion syndrome)

59
Q

What is the usual treatment for immunodeficiencies?

A

IVIG