Autoimmunity (Exam 2)

Question 1

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

Answer 1

self-tolerance

Question 2

What can a loss of self-tolerance result in?

Answer 2

autoimmunity

Question 3

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

Answer 3

central tolerance

Question 4

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.

Answer 4

peripheral tolerance

Question 5

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

Answer 5

molecular mimicry

Question 6

How can the MHC molecular structure affect or cause autoimmunity?

Answer 6

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.

Question 7

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

Answer 7

People of the same family

Question 8

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

Answer 8

Identical twins

Question 9

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

Answer 9

The MHC gene

Question 10

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

Answer 10

systemic

Question 11

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

Answer 11

systemic

Question 12

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

Answer 12

organ specific

Question 13

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

Answer 13

organ specific

Question 14

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

Answer 14

organ specific

Question 15

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

Answer 15

organ specific

Question 16

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

Answer 16

Effects of nonspecific involvement (of SLE)

Question 17

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

Answer 17

Effects of joint involvement (of SLE)

Question 18

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.

Answer 18

Effects of uncommon appearance (of SLE)

Question 19

The most common cause of death in patients with SLE

Answer 19

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

Question 20

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.

Answer 20

The patient has SLE

Question 21

A specific state of unresponsiveness to antigens is known as…

Answer 21

Anergy

Question 22

What percentage of the population is affected by autoimmunity?

Answer 22

5-7%

Question 23

When does central tolerance take place?

Answer 23

When B cells mature in the bone marrow.

Question 24

When does peripheral tolerance take place?

Answer 24

After T and B cells mature and enter periphery

Question 25

The following defects are all caused by what? Abnormal expression, dysfunction of signaling molecules, dysregulation of cytokines, Fc receptor polymorphisms.

Answer 25

B-cell defects

Question 26

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

Answer 26

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

Question 27

Fluorescent anti-nuclear antibody

Answer 27

FANA

Question 28

Extractable nuclear antigen

Answer 28

ENA

Question 29

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

Answer 29

Profile testing

Question 30

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

Answer 30

Homogenous patten

Question 31

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

Answer 31

Peripheral pattern

Question 32

Pattern with large speckled covering the whole nucleoplasm.

Answer 32

Speckled pattern

Question 33

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

Answer 33

Nucleolar

Question 34

What two autoimmune disorders are ENAs associated with?

Answer 34

Mixed Connective Disease and SLE

Question 35

What is peak onset age for SLE?

Answer 35

20-40 yo

Question 36

Is SLE more common in men or women?

Answer 36

Women

Question 37

What HLA isotypes are associated with SLE?

Answer 37

HLA-DR and HLA-DQ

Question 38

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

Answer 38

ENA

Question 39

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

Answer 39

B cells increase and T cells decrease

Question 40

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

Answer 40

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

Question 41

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

Answer 41

Anti-DNA

Question 42

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

Answer 42

Anti-RNP, Anti-SSA/SSB

Question 43

What is the typical onset age for RA?

Answer 43

between 35-50 yo

Question 44

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

Answer 44

3 times

Question 45

What genes and alleles are specifically associated with RA?

Answer 45

MHCII genes and DR4 alleles

Question 46

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

Answer 46

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

Question 47

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

Answer 47

Extractable nuclear antigen (ENA) assay

Question 48

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?

Answer 48

Fluorescent antinuclear antibody (FANA)

Question 49

Malaise, fever, weight loss, and transient joint pain

Answer 49

Effects of non-specific involvement (of RA)

Question 50

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

Answer 50

Effects of join-involvement (of RA)

Question 51

IgM antibody against the Fc portion of the IgG molecule

Answer 51

Rheumatoid factor (RF)

Question 52

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

Answer 52

Cardiovascular disease

Question 53

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

Answer 53

Systemic effects of RA

Question 54

How does RF play a role in pathogenesis of RA?

Answer 54

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

Question 55

Antibody to cyclic citrullinated proteins

Answer 55

Anti-CCP

Question 56

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

Answer 56

Anti-CCP

Question 57

How do labs test for RF?

Answer 57

Latex agglutination test; agglutination occurs if RF is present

Question 58

How do labs test for anti-CCP?

Answer 58

ELISA

Question 59

What are the three main forms of treatment for RA?

Answer 59

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

Question 60

What ANA pattern is often observed for patients with RA?

Answer 60

Speckled pattern; typically directed against ribonucleoprotein

Question 61

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

Answer 61

They can yield false negatives and false positives

Question 62

What are the common autoantobodies for Sjogren's syndrome?

Answer 62

anti-SS-B and anti-SS-B

Question 63

Are patients with Sjogen's RF positive?

Answer 63

Yes

Question 64

Is Sjogen's more common in women or men

Answer 64

90% more common in women

Question 65

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

Answer 65

Sjogren's syndrome

Question 66

What are the common autoantibodies of Scleroderma?

Answer 66

Anti-Scl-70

Question 67

What are the common autoantibodies of scleroderma?

Answer 67

Anti-Scl-70

Question 68

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.

Answer 68

Scleroderma

Question 69

What is the ANA pattern for scleroderma?

Answer 69

Speckled pattern and centromere pattern

Question 70

Are patients with scleroderma RF positive?

Answer 70

Yes

Question 71

What disorder are the specific symptoms describing? ``` Calcinosis Raynaud phenomenon Esophageal dysfunction Sclerodactyly Telangiectasia ```

Answer 71

CREST syndrome, a limited form of scleroderma

Question 72

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.

Answer 72

Myasthenia Gravis

Question 73

What autoantibodies are associated with Myasthenia Gravis?

Answer 73

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

Question 74

What autoantibodies are associated with Goodpasture's syndrome?

Answer 74

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

Question 75

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

Answer 75

Goodpasture's syndrome