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Immunology Exam 4 > Autoimmunity > Flashcards

Autoimmunity Flashcards

1
Q

What is the approximate percentage of the world’s population affected with autoimmune disease?

A

5 percent

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2
Q

Where does central tolerance occur?

A

The B-cell and the T-cell

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3
Q

What happens when self tolerance mechanisms fail?

A

Auto-immune disease

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4
Q

Autoimmune disease involves both B and T cells. True or False?

A

True

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5
Q

Autoimmunity mechanisms include those for types 1,2,3 and 4. True or False

A

False, all but type 1

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6
Q

What is epitope spreading?

A

In response to an original insult, the inflammatory response causes damage, unveils a new epitope against which an immune reaction can be mounted

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7
Q

Autoimmunity is caused mainly be genetic factor, True or false

A

False, autoimmunity is caused by environmental factors as well

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8
Q

An example of an autoimmune disease with strong genetic component is _

A

Diabetes Mellitus type 1

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9
Q

The strongest genetic association with autoimmune disease are with _. Genes that predispose to autoimmune disease are usually involved with _

A

MHC genes

Establishing self tolerance

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10
Q

Majority of autoimmune diseases are caused by a single gene defect. True or false

A

False. These are the minority

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11
Q

What is the function of AIRE?

A

Required to express and present self antigens in the thymus. No AIRE, no self presentation

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12
Q

What disease results from defective AIRE?

A

APECED. Autoimmune PolyEndocrinopathy, Candidiasis, Ectodermal Dystropy

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13
Q

What are the symptoms of AIRE?

A

Hypoparathyroidism
Adrenal Insufficiency
Hypogonadism
(i.e. Multiple endocrine disorders)

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14
Q

The major function of FoxP3 is _

A

Major transcription factor for regulatory T-cells

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15
Q

Non-functional FoxP3 leads to _. What are the symptoms?

A

IPEX (Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked)

  • Neonatal diabetes 1, throiditis, diarrhea, dermatitis
  • Fatal in year 1
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16
Q

What is FAS?

A

The major apoptosis ligand

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17
Q

What disease occurs if FAS is mutated?

A

ALPS (Autoimmune Lymphoproliferative Syndome)

- Can turn on immune system fine, but can not turn off when no longer sick - Lymphadenopathy

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18
Q

What is bystander effect?

A

APCs activate T-cells in the vicinity of the immune reaction. These T cells can cause disease if specific to self

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19
Q

What is molecular mimicry?

A

Where an antibody to a pathogen ends up recognizing self because of similarity between the 2 proteins

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20
Q

SLE is a type _ autoimmune disease? It is most prevalent in what population?

A 
Type III (antibody - antigen deposition all over)
More common in females
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21
Q

What genetic factor are implicated in SLE?

A

Several, including
Complement deficiencies
HLA - DR2/DR3

22
Q

The disease mechanism for SLE is _? What organs are affected?

A

Ab/Ag deposition all over the body

Virtually all organs are affected

23
Q

2 specific type of Abs are observed in lupus. They are?

A

Anti-nuclear Ab

Anti-dsDNA Ab (correlates with disease severity)

24
Q

Rash, arthritis, glomerulonephritis, vasculitis, hemolytic anemia, thrombocytopenia, CNS involvement are all associated with _

A

Lupus

25
Q

Treatment of lupus involves _

A

Immuno-suppressive medications

26
Q

There are 11 diagnostic criteria related to Lupus. How many have to be met for diagnosis?

A

4/11

27
Q

RA is the most common inflammatory arthritis. What are 2 risk factors?

A

Cigarette smoking

HLA DR4

28
Q

The disease mechanism of RA involves _

A

T-cells and antibodies

Arginine residues converted to citrulline

29
Q

What are the cytokines involved in RA?

A

IL17, TNA, IL1

30
Q

The clinical features of RA include _ (2)

A

Symmetrical inflammation of small and large joints

Synovitis

31
Q

Diagnostic tests for RA include _ (2)

A 
Rheumatoid factor (IgM against patients IgG)
Anti-cyclic citrulline (Anti-CCP)
32
Q

Treatment of RA involves? (2)

A

Glucocorticoids

DMARDs (Disease Modifying Anti-Rheumatic Drugs)

33
Q

Diabetes Mellitus Type 1 is a multisystem metabolic disease. It is characterized by _ distribution

A

Bimodal (4-6 and 10-14)

34
Q

The disease mechanism of DM type 1 is _. This is mediated by what?

A

Destruction of pancreatic islet cells

CD4 TH1 cells

35
Q

Why are anti-inflammatory drugs not used for DM type 1?

A

The damage is already done. No inflammation to treat

36
Q

What are the clinical features of DM type 1? (3)

A 
Hyperglycemia / Ketoacidosis
Macrovascular complications (atherosclerosis, ischemic necrosis)
Microvascular complications (retina, glomeruli)
37
Q

The most common disease of neuromuscular transmission is _

A

Myesthesia Gravis disease

38
Q

The disease mechanism if Myesthesia Gravis is _

A

Abs against the AchR in the neuromuscular junction. Act as antagonists, cause muscles weakness

39
Q

What type of hypersensitivity is Myesthesia Gravis?

A

Type 2

40
Q

What type of tumor is usually found along with Myesthesia Gravis? What usually happens when these tumors are removed?

A

Thymoma

Patients get better

41
Q

Myesthesia Gravis is associated with these clinical features _

A

Muscle weakness, fatiguability with muscle use

42
Q

What is myesthenic crisis?

A

When the muscle weakness extends to respiratory muscles, leading to respiratory failure

43
Q

How is myesthenia gravis treated? (4)

A

Anticholinesterase agents
Immunosuppression
Immunotherapy (plasma exchange)
Thymectomy

44
Q

What is Graves disease?

A

Thyroid stimulated by anti-TSH receptor (agonists)

45
Q

An aspect of Graves disease that makes it dissimilar from other autoimmune disease is absence of _

A

Inflammation

46
Q

Graves disease is characterized by Hyperthyroidism. Symptoms include _ (6)

A 
Anxiety 
Tremor
Palpitation
Heat intolerance
Weight loss
Proptosis and periorbital swelling
47
Q

What is Hashimoto’s thyroiditis?

A

An autoimmune destruction of the thyroid gland, leading to hypothyroidism

48
Q

Symptoms are opposite of hyperthyroidism in graves disease.

A

Yes

49
Q

Treatment of hypothyroidism is _

A

Thyroid hormone replacement

50
Q

Why don’t the anti-AchR receptors in the Graves disease affect the AchRs in the CNS?

A

Different subtypes in both locations.

Immunology Exam 4 (7 decks)

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