Autoimmunity

Question 1

autoimmunity:

-immune reactions directed at ____

Answer 1

self-antigens

Question 2

autoimmunity:

-acute and/or chronic _____ and _____

Answer 2

inflammation;

cell injury

Question 3

autoimmunity:

severity ranges from _____ to _____

Answer 3

minor;

lethal

Question 4

autoimmunity:

cures

Answer 4

no cure for most

Question 5

allergy: _____ antigen and _____ duration

Answer 5

environmental;

limited

Question 6

allergies ____ with removal of environmental antigen

Answer 6

resolve

Question 7

autoimmunity: _____ antigen and _____ duration

Answer 7

environmental or endogenous;

chronic

Question 8

autoimmunity ____ with removal of environmental antigen

Answer 8

does not resolve

Question 9

autoimmunity is largely limited to type ____ hypersenstivities

Answer 9

II - IV

Question 10

most affected organs and tissues

Answer 10

• bone marrow
• lining of the joints
• blood vessels
• connective tissues
• endocrine glands
• kidneys
• muscles
• skin

Question 11

steps to autoimmunity

Answer 11

• “normal” autoimmunity
• multigenic
• hormonal
• environmental triggers (infections, drug-induced, toxins)
• breakdown of tolerance

Question 12

there are some ____ that predispose people to autoimmune diseases

Answer 12

alleles

Question 13

uveitis is associated with ____

Answer 13

HLA B27

Question 14

uveitis:

• inflammation of ____
• etiology:

Answer 14

uvea;

idiopathic, infection, injury, or autoimmune disorders

Question 15

breakdown of central and peripheral tolerance:

-faulty ____

Answer 15

clonal deletion (auto-reactive lymphocytes allowed to mature and enter circulation)

Question 16

breakdown of central and peripheral tolerance:

-loss of ____

Answer 16

Treg-cells (hyper-responsive T and B-lymphocytes; prolonged immune reactions)

Question 17

breakdown of central and peripheral tolerance:

abnormal expression of _____

Answer 17

HLA class II molecules

Question 18

antigens in autoimmunity:

sequestered antigens

Answer 18

immunoprivileged organs

Question 19

antigens in autoimmunity:

infectious disease

Answer 19

• molecular mimicry

- original insult

Question 20

tissue-specific, antibody-mediated, autoimmune diseases (Type II)

Answer 20

• auto-antibodies direct at auto-antigens
• affect normal function of the target organ
• induce tissue damage
• auto-Ab levels increase with age in healthy adults

Question 21

rheumatic fever

Answer 21

• Type II
• strep infection induces cross-reactive auto-Abs
• molecular mimicry
• protein in wall of strep bacteria looks similar to antigens of tissues in our heart and myocardium - auto-Abs attack our heart

Question 22

pernicious anemia

Answer 22

• Type II

- auto-Abs block transport molecule in GI tract

Question 23

myasthenia gravis

Answer 23

• Type II
• auto-Abs block muscle function
• prevent ACh from binding, no muscle contraction, paralysis

Question 24

graves disease

Answer 24

• Type II
• auto-Abs stimulate thyroid gland to produce more thyroid hormone
• have low levels of TSH and high levels of thyroid hormone

Question 25

ocular manifestations of myasthenia gravis

Answer 25

- diplopia - ptosis - blurred vision

Question 26

ocular manifestations of graves disease

Answer 26

- proptosis - periorbital edema - abnormal EOMs - exposure keratopathy - optic nerve compression

Question 27

autoimmune complex-mediated diseases (Type III)

Answer 27

- auto-Ab:Ag immune complexes deposit throughout body - infectious initiation (reactive arthritis, poststreptococcal glomerulonephritis) - undetermined initiation (lupus, rheumatoid arthritis (also type IV))

Question 28

cell-mediated autoimmunity (Type IV)

Answer 28

- does not involve antibodies - APC activates Th cell, which activates Tc cell - Tc cells use perforin and granzymes to attack cells - Th cell will increase amount of cytokines it produces, including interferon gamma, which increases macrophage activity

Question 29

cell-mediated autoimmune diseases (type IV)

Answer 29

- rheumatoid arthritis (targets multiple cell types) - Crohn's disease (targets epithelial cells of GI tract) - multiple sclerosis (targets oligodendrocytes in CNS) - type I diabetes (targets beta cells in pancreas)

Question 30

organ-specific autoimmune diseases

Answer 30

- directed against a unique antigen in a given organ - type II hypersensitivity (myasthenia gravis, Hashimoto's thyroiditis, pernicious anemia) - type IV hypersensitivity (Crohn's disease, type I diabetes)

Question 31

systemic autoimmune diseases

Answer 31

- wide range of antigens - widespread damage - general defect in immune regulation - examples: lupus, MS, scleroderma - may be types II, II, or IV, or combination

Question 32

systemic lupus erythematosus epidemiology

Answer 32

- chronic, multisystem inflammatory disease - type III hypersensitivity - 90% of cases in women between 20-40 - female:male 10:1 - black:white 8:1

Question 33

pathogenesis of SLE

Answer 33

- DNA and histones released from cells - phagocytized by APCs - activate T-cells - activate B-cells - produce antibodies against nuclear antigens

Question 34

SLE target organs

Answer 34

- joints - blood vessels - kidneys - heart - skin - lungs - brain

Question 35

SLE auto-antibodies against:

Answer 35

- nucleic acids - erythrocytes - lymphocytes - platelets - coagulation proteins - phospholipids

Question 36

11 common symptoms of SLE

Answer 36

- facial (malar) rash - discoid rash - photosensitivity - oral or nasopharyngeal ulcers - non-erosive arthritis - serositis - renal disorders - neurologic disorders - hematologic disorders - immunologic disorders - presence of anti-nuclear antibodies (ANA)

Question 37

ocular manifestations of SLE (present in ____ of patients)

Answer 37

50%; - secondary Sjogren syndrome - uveitis (rare) - episcleritis/scleritis - neuro-ophthalmic lesions - retinal vasculopathy (cotton wool spots, most common) - bulls-eye maculopathy

Question 38

Sjogren's syndrome epidemiology

Answer 38

- targets salivary and lacrimal glands - ~4 million affected in US - 40-60 years of age - female:male 9:1 - primary and secondary forms

Question 39

primary Sjogren's syndrome

Answer 39

lymphocyte infiltration of salivary and lacrimal glands (cause damage and reduce function)

Question 40

secondary Sjogren's syndrome

Answer 40

immune-complex accumulation in salivary and lacrimal glands (causes inflammation and reduced function)

Question 41

Sjogren's syndrome pathogenesis

Answer 41

- systemic autoimmune disease: RA and SLE - lymphocytic infiltrations - auto-antibodies play a role