Autoimmunity (Exam 2) Flashcards

1
Q

The ability of the immune system to accept self-antigens and not initiate a response against them.

A

self-tolerance

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2
Q

What can a loss of self-tolerance result in?

A

autoimmunity

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3
Q

Tolerance that occurs in primary or central lymphoid organs and is also called negative selection of self-reactive lymphocytes.

A

central tolerance

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4
Q

Lymphocytes that recognize self-antigens in secondary lymphoid organs are rendered incapable of reacting with those organs. This type of immunological tolerance is where self-reactive lymphocytes are terminated to avoid an autoimmune disorder.

A

peripheral tolerance

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5
Q

The fact that many bacterial or viral agents have antigens that closely resemble the structure or amino acid sequence of self-antigens.

A

molecular mimicry

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6
Q

How can the MHC molecular structure affect or cause autoimmunity?

A

If a self-antigen is able to bind to the peptide cleft of the molecule, it will be presented by the MHC to killer T cells, therefore causing autoimmunity.

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7
Q

Is autoimmunity more prevalent among people of the same family or among unrelated people?

A

People of the same family

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8
Q

Is autoimmunity more prevalent among identical twins (monozygotic) or non-identical (dizygotic) twins/siblings?

A

Identical twins

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9
Q

What gene is heavily focused on in the genetic research of autoimmunity?

A

The MHC gene

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10
Q

Is SLE an example of a systemic or organ specific autoimmune disease?

A

systemic

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11
Q

Is RA an example of a systemic or organ specific autoimmune disease?

A

systemic

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12
Q

Is Goodpasture’s syndrome an example of a systemic or organ specific autoimmune disease?

A

organ specific

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13
Q

Is myasthenia gravis an example of a systemic or organ specific autoimmune disease?

A

organ specific

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14
Q

Is scleroderma an example of a systemic or organ specific autoimmune disease?

A

organ specific

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15
Q

Is Sjogren’s syndrome an example of a systemic or organ specific autoimmune disease?

A

organ specific

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16
Q

Fatigue, weight loss, malaise, fever and anorexia are among the first to appear.

A

Effects of nonspecific involvement (of SLE)

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17
Q

The most frequently reported manifestation (over 90% of patients); Polyarthralgia and typically symmetric arthritis in small joints of the hands, wrists, and knees.

A

Effects of joint involvement (of SLE)

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18
Q

The second most frequently occurring manifestation (approx. 80% of patients); Skin manifestations including an erythematous rash in areas contacted by UV and the classic butterfly rash on the face.

A

Effects of uncommon appearance (of SLE)

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19
Q

The most common cause of death in patients with SLE

A

The deposition of immune complexes in kidney tissue leading to renal failure

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20
Q

Patients with this disorder may experience any of the following systemic effects: renal failure, cardiac issues (tachycardia, pericarditis, or ventricular enlargement), pleuritis, neuropsychiatric manifestations, and hematologic abnormalities.

A

The patient has SLE

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21
Q

A specific state of unresponsiveness to antigens is known as…

A

Anergy

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22
Q

What percentage of the population is affected by autoimmunity?

A

5-7%

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23
Q

When does central tolerance take place?

A

When B cells mature in the bone marrow.

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24
Q

When does peripheral tolerance take place?

A

After T and B cells mature and enter periphery

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25
Q

The following defects are all caused by what?

Abnormal expression, dysfunction of signaling molecules, dysregulation of cytokines, Fc receptor polymorphisms.

A

B-cell defects

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26
Q

Defects of polyclonal B-cell activation may be enhanced by…

A

Gran-negative bacteria and many viruses (ex. CMV and EBV)

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27
Q

Fluorescent anti-nuclear antibody

A

FANA

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28
Q

Extractable nuclear antigen

A

ENA

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29
Q

If an FANA result is positive, what is the next step to be completed?

A

Profile testing

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30
Q

Pattern with a smooth, even staining or the nucleus with or without apparent masking of the nucleoli.

A

Homogenous patten

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31
Q

Pattern with fluorescence most intensely concentrated at the periphery of the nucleus with a large ring starting from the internal nuclear membrane.

A

Peripheral pattern

32
Q

Pattern with large speckled covering the whole nucleoplasm.

A

Speckled pattern

33
Q

Pattern with 23 or 46 bright speckles or void granules spread over the nucleus of interphase cells.

A

Nucleolar

34
Q

What two autoimmune disorders are ENAs associated with?

A

Mixed Connective Disease and SLE

35
Q

What is peak onset age for SLE?

A

20-40 yo

36
Q

Is SLE more common in men or women?

A

Women

37
Q

What HLA isotypes are associated with SLE?

A

HLA-DR and HLA-DQ

38
Q

Which types of assay has the higher specificity for antigens, ENA or FANA?

A

ENA

39
Q

What happens to B and T cells in a patient with SLE?

A

B cells increase and T cells decrease

40
Q

A homogenous pattern in SLE diagnosis shows evidence of which autoantibodies?

A

Anti-DNA, Anti-DNP, and Anti-histone

41
Q

A peripheral pattern in SLE diagnosis shows evidence of which type of autoantibodies?

A

Anti-DNA

42
Q

A speckled pattern in SLE diagnosis shows evidence of which type of autoantibodies?

A

Anti-RNP, Anti-SSA/SSB

43
Q

What is the typical onset age for RA?

A

between 35-50 yo

44
Q

How many times more likely are women to get RA than men?

A

3 times

45
Q

What genes and alleles are specifically associated with RA?

A

MHCII genes and DR4 alleles

46
Q

What are the three most common forms of treatment for SLE?

A

Antimalarials or topical steroids, High dose of aspirin or anti-inflammatory, Systemic corticosteroids

47
Q

Which immunoassay detects presence of autoantibodies in the blood by using six extractable nuclear proteins?

A

Extractable nuclear antigen (ENA) assay

48
Q

Which immunoassay detects the presence of ANAs in patient serum by adding the serum to a microscopic slide fixed with animal or human cells, doing a wash step, and then adding fluorescent tagged anti-human Ag?

A

Fluorescent antinuclear antibody (FANA)

49
Q

Malaise, fever, weight loss, and transient joint pain

A

Effects of non-specific involvement (of RA)

50
Q

Symmetrical affects to the joints; stiffness and pain in joints that is worse in the morning and gets better throughout the day; joint pain starting in smaller joints and spreading into larger joints such as knees, hips, cervical spine, and shoulders; osteoporosis in 20-30% of patients; joint pain that leads to muscles spasms and limited range of motion

A

Effects of join-involvement (of RA)

51
Q

IgM antibody against the Fc portion of the IgG molecule

A

Rheumatoid factor (RF)

52
Q

What is the most common cause of death in patients with RA?

A

Cardiovascular disease

53
Q

Formation of subcutaneous nodules, pericarditis, lymphadenopathy, splenomegaly, interstitial lung disease, or vasculitis; development of nodules over bones; development of Sjogren’s syndrome or Fetyl’s syndrome; cardiovascular disease

A

Systemic effects of RA

54
Q

How does RF play a role in pathogenesis of RA?

A

It increases macrophage activity and enhances antigen presentation to T cells by APCs

55
Q

Antibody to cyclic citrullinated proteins

A

Anti-CCP

56
Q

The lead marker for detection of RA, more specific than the other

A

Anti-CCP

57
Q

How do labs test for RF?

A

Latex agglutination test; agglutination occurs if RF is present

58
Q

How do labs test for anti-CCP?

A

ELISA

59
Q

What are the three main forms of treatment for RA?

A

Non-steroidal anti-inflammatory drugs (NSAIDs), Anti-rheumatic drugs, and joint replacement

60
Q

What ANA pattern is often observed for patients with RA?

A

Speckled pattern; typically directed against ribonucleoprotein

61
Q

What is a limitation of screening tests for RF and anti-CCP?

A

They can yield false negatives and false positives

62
Q

What are the common autoantobodies for Sjogren’s syndrome?

A

anti-SS-B and anti-SS-B

63
Q

Are patients with Sjogen’s RF positive?

A

Yes

64
Q

Is Sjogen’s more common in women or men

A

90% more common in women

65
Q

What disorder is described by the following symptoms?

Inflammation of the salivary and lacrimal glands leading to dryness of the eyes and mouth, joint pain, vaginal dryness leading to yeast infections, persistent dry cough, and prolonged fatigue

A

Sjogren’s syndrome

66
Q

What are the common autoantibodies of Scleroderma?

A

Anti-Scl-70

67
Q

What are the common autoantibodies of scleroderma?

A

Anti-Scl-70

68
Q

What disorder is described by the following symptoms?

Thickening and tightening of skin caused by excessive deposits of collagen, petechiae, reddish/scaly skin, and visible blood vessels.

A

Scleroderma

69
Q

What is the ANA pattern for scleroderma?

A

Speckled pattern and centromere pattern

70
Q

Are patients with scleroderma RF positive?

A

Yes

71
Q

What disorder are the specific symptoms describing?

Calcinosis 
Raynaud phenomenon
Esophageal dysfunction 
Sclerodactyly 
Telangiectasia
A

CREST syndrome, a limited form of scleroderma

72
Q

What disorder is described by the following symptoms?

Weakening of the skeletal muscle due to damage to the acetylcholine receptors, facial weakness, drooping of eyelids, and difficulty swallowing.

A

Myasthenia Gravis

73
Q

What autoantibodies are associated with Myasthenia Gravis?

A

Anti-AChR (binding, blocking, disrupting)
Anti-MuSK (muscle kinase)
Anti-LRP4 (lipoprotein)

74
Q

What autoantibodies are associated with Goodpasture’s syndrome?

A

Autoantibody to the glomerular, renal tubular and alveolar basement membranes (renal and pulmonary basement membranes)

75
Q

What disorder is described by the following symptoms?

Foamy, bloody or dark colored urine, decreased urine output, cough with bloody sputum, difficulty breathing after exertion, weakness, fatigue, nausea/vomiting, weight loss, nonspecific chest pain, and pale skin

A

Goodpasture’s syndrome