AUTOIMMUNITY

Question 1

It is characterized by the presence of an autoantibody to an antigen in the basement membranes of glomeruli and alveoli of the lungs.

Answer 1

good pasture syndrome

Question 2

70% - 80% of patients carry the HLA-DRB1-15 antigen

Answer 2

good pasture syndrome

Question 3

Treatment of Good Pasture Syndrome

Answer 3

corticosteroids - to stop inflammation
plasmapheresis
lifetime hemodialysis or kidney transplantation

Question 4

an autoimmune disease that affects neuromuscular junction characterized by weakness and fatigability of skeletal muscles, causes antibody-mediated damage to acetylcholine receptors in skeletal muscles

Answer 4

myasthenia gravis (MG)

Question 5

HLA haplotype, A1,B8, DR3 has strong association with _ while HLA antigens B7 & DR2 are more likely to appear in _

Answer 5

Early Onset MG; Late Onset MG

Question 6

It is a radio-labeled snake venom used to detect antibodies to ACHR in radioimmunoprecipitation (RIPA)

Answer 6

a-bungaro-toxin

Question 7

an autoimmune disorder involving inflammation and destruction of the CNS

Answer 7

Multiple Sclerosis

Question 8

Multiple Sclerosis is most closely associated with the inheritance of this particular HLA molecule

Answer 8

HLA-DRB1*1501

Question 9

these are the environmental factors that appear to be associated with multiple sclerosis

Answer 9

• reduced exposure to sunlight
• vitamin D deficiency
• cigarette smoking

Question 10

these are alleles associated with a higher risk of developing autoimmune hepatitis (AIH) or chronic active hepatitis

Answer 10

HLA-DRB1 & HLA-DQB1

Question 11

What are the two types of Autoimmune Hepatitis (AIH) that can be differentiated on the basis of its autoantibody specificity

Answer 11

• AIH-1 - 2/3 of all AIH cases, F:M of 4:1
• AIH 2 - seen mostly in children and has F:M ratio of 10:1

Question 12

This is necessary to confirm the diagnosis of Autoimmune Hepatitis and to assess the extent of liver damage

Answer 12

liver biopsy

Question 13

It has a W:M ratio of 10:1 and it is the most common autoimmune liver disease

Answer 13

primary biliary cirrhosis (PBC)

Question 14

treatment for primary biliary cirrhosis

Answer 14

ursodeoxycholic acid (UDCA) , a bile acid that helps move bile through liver; liver transplant

Question 15

An autoimmune disorder associated with an environmental trigger - dietary gluten

Answer 15

Celiac Disease

Question 16

In lab diagnosis of celiac disease, it is a serological method of choice for initial testing which is ELISA based

Answer 16

tissue transglutaminase (tTG)

Question 17

An autoimmune disease affecting small intestines and other organs, HLA-DQ2 (90-95%) or HLA-DQ8, W:M of 2-3:1

Answer 17

celiac disease

Question 18

Type 1 Diabetes carries these HLA genes

Answer 18

HLA-DR3 and DR4 genes

Question 19

an endocrine disorder characterized by hyperglycemia (a high level of glucose in the blood); characterized by a complete or nearly complete deficiency in insulin

Answer 19

Type 1 Diabetes

Question 20

hyperglycemia does not become evident until ?

Answer 20

80% or more of the beta cells are destroyed

Question 21

Hashimoto’s thyroiditis is associated with the inheritance of these HLA antigens

Answer 21

HLA antigens DR3, DR4, DR5, and DQ7

Question 22

Graves dse is associated with this HLA antigen

Answer 22

HLA-DR3

Question 23

aka chronic lymphocytic thyroiditis; Japan, 1912; Dr. Hakaru Hashimoto, most common autoimmune disease, 8 in evey 1,000; W:M-5-10:1

Answer 23

Hashimoto’s Thyroiditis

Question 24

autoantibodies that are produced against proteins that are present in the neutrophil granules

Answer 24

Antineutrophil Cytoplasmic Antibodies

Question 24

strongly associated with three syndromes involving vascular inflammation WG, MPA, EGPA

Answer 24

Antineutrophil Cytoplasmic Antibodies

Question 25

a rare autoimmune disease involving inflammation of the small- to medium-sized blood vessels, or vasculitis, mainly directed against the PR3 antigen

Answer 25

Granulomatosis With Polyangiitis (Wegener’s Granulomatosis)

Question 26

what is the antibody that is most often tested to aid in making the initial diagnosis of RA

Answer 26

Rheumatoid Factor (RF)

Question 27

* affects about 0.5% to 1.0% of the adult population; age:25 and 55; W:M-3:1 * can be characterized as a chronic, symmetric, and erosive arthritis of the peripheral joints that can also affect multiple organs such as the heart and the lungs ∟ HLA-DRB1 alleles or PTPN22 gene polymorphisms ∟ strongest environmental risk factor - cigarette smoking

Answer 27

Rheumatoid Arthritis

Question 28

* associated with deep-vein and arterial thrombosis and with recurrent pregnancy loss * found in up to 60% of patients with lupus * can be identified by their ability to cause false-positive results in nontreponemal tests for syphilis

Answer 28

Antiphospholipid antibodies

Question 29

autoantibodies that are directed against antigens in the nuclei of mammalian cells

Answer 29

Anti-Nuclear Antibodies

Question 30

* chronic systemic inflammatory disease * peak age of onset: 20-40 yo, * Women:Men – 9:1 * 5 yr survival rate - 90% (today) ∟ genetic defects in genes coding for HLA-A1, B8, and DR3 — increases the chance of developing lupus

Answer 30

Systemic Lupus Erythematosus (SLE)

Question 31

Immune Response is targeted toward self-antigens resulting in organ and tissue damage

Answer 31

Autoimmune Disease

Question 32

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

Answer 32

self-tolerance

Question 33

2 levels of Immunologic Tolerance

Answer 33

central & peripheral tolerance

Question 34

a state of immune unresponsiveness that is directed against a self-antigen

Answer 34

Immunologic Tolerance

Question 35

more prevalent among family members and among monozygotic (genetically identical) twins than dizygotic (non-identical) twins or siblings

Answer 35

Genetics

Question 36

Enumerate other Endogenous and Environmental Factors

Answer 36

hormonal influence, tissue trauma and release of cryptic antigens, microbial infections

Question 37

Enumerate 5 mechanisms of microbes

Answer 37

1) molecular mimicry 2) bystander effect 3) superantigens 4) Epigenetics and Modification of Self-Antigens 5) Interactions between factors

Question 38

Superantigens that can cause polyclonal activation of B cells

Answer 38

Epstein-Barr virus (EBV) and cytomegalovirus (CMV)

Question 39

the microorganism can induce a local inflammatory response that recruits leukocytes and stimulates APCs to release cytokines that nonspecifically activate T cells

Answer 39

Bystander Effect

Question 40

many bacterial or viral agents contain antigens that closely resemble the structure or amino acid sequence of self-antigens, give an example of a bacteria

Answer 40

molecular mimicry, S. pyogenes

Question 41

proteins produced by various microbes that have the ability to bind to both class II MHC molecules and TCRs, regardless of their antigen specificity

Answer 41

Superantigens

Question 42

refers to modifications in gene expression that are not caused by changes in the original DNA sequence

Answer 42

Epigenetics

Question 43

Triggered by exposure to environmental toxins, ingestion of harmful foods or drugs, or the aging process

Answer 43

Epigenetics and Modification of Self-Antigens

Question 44

citrullination of collagen

Answer 44

Rheumatoid Arthritis

Question 45

glycosylation of myelin

Answer 45

Multiple Sclerosis

Question 46

complex interactions between genetic and environmental factors may cause break in immunologic tolerance

Answer 46

Interactions Between Factors

Question 47

has genetic defects in genes coding for HLA-A1, B8, and DR3

Answer 47

SLE

Question 48

antibodies to endothelial cells

Answer 48

vasculitis

Question 49

can cross the placenta and have been associated with neonatal lupus

Answer 49

Antibodies to both SS-A/Ro and SS-B/La