Immunology: Test 2: 4/5. Autoimmune I&ii

Front 1

What does a direct Coombs' test show?

Back 1

IgG

Front 2

Autoimmune disease: Systemic lupus erythematosus


please list affected organs/tissues

Back 2

Systemic, joints

Front 3

Autoimmune disease: Rheumatoid arthritis


please list affected organs/tissues

Back 3

Joints, systemic

Front 4

Autoimmune disease: Insulin-dependent diabetes


please list affected organs/tissues

Back 4

Pancreas

Front 5

Autoimmune disease: Thyroiditis


please list affected organs/tissues

Back 5

Thyroid

Front 6

Autoimmune disease: Addison’s Disease


please list affected organs/tissues

Back 6

Adrenals

Front 7

Autoimmune disease: Polyglandular autoimmune
syndromes


please list affected organs/tissues

Back 7

Various endocrine glands

Front 8

Autoimmune disease: Graves Disease




please list affected organs/tissues

Back 8

Thyroid

Front 9

Autoimmune disease: Goodpasture’s


please list affected organs/tissues

Back 9

Kidneys and lungs

Front 10

Autoimmune disease Inflammatory bowel disease


please list affected organs/tissues

Back 10

Intestinal mucosa

Front 11

Autoimmune disease:
Immune hemolytic anemia


please list affected organs/tissues

Back 11

Erythrocytes

Front 12

Autoimmune disease:
Myasthenia gravis


please list affected organs/tissues

Back 12

Muscles

Front 13

Autoimmune disease:
Vasculitis


please list affected organs/tissues

Back 13

Blood vessels

Front 14

Autoimmune disease:
Pemphigus


please list affected organs/tissues

Back 14

Skin

Front 15

Autoimmune disease:
Multiple sclerosis


please list affected organs/tissues

Back 15

Central nervous system

Front 16

What is immunologic tolerance?

Back 16

Refers to a state of unresponsiveness which is specific for a particular antigen, and is induced by prior exposure to that antigen.

The most important form of tolerance is “self- tolerance” to prevent autoimmune responses.

Front 17

What are the 3 aspects of tolerance induction/maintenance

Back 17

Clonal deletion
Clonal anergy
Suppression

Front 18

What is Central Tolerance?

Back 18

removal of self reactive T cells in the thymus and B cells in the bone marrow

Front 19

Clonal deletion

Back 19

removal of self-reacting lymphocytes (thymic selection)

Front 20

Clonal anergy

Back 20

the self-reactive clone is present but will not respond for some reason (e.g., lack of second signal)

Front 21

Suppression

Back 21

- inhibiting cellular activation or effector function, via interaction with cells or suppressive cytokines (e.g., TGF-)

Front 22

costimulatory signals

Back 22

if there is no costimulatory signal, antigen is more likely to signal a cell to die or to be anergic

Front 23

summarize what occurs in the thymus

Back 23

THE THYMUS SELECTS T CELLS WITH RECEPTORS THAT BIND TO ANTIGENIC PEPTIDES, ASSOCIATED WITH MHC MOLECULES, BUT DELETES CELLS WITH HIGH AFFINITY FOR "SELF" ANTIGENS

Front 24

Are there any mature lymphoid cells in the normal adult that can react to self antigens?
If the answer is “yes”, how are they controlled?

Back 24

yes

they are controlled by tolerance: suppression, anergy, clonal deletion

Front 25

What are the 5 major features of Systemic Lupus Erythematosus (SLE)

Back 25

1. Presence of Anti-nuclear antibody (ANA)
2.Antibody against double stranded DNA******
3. Increased serum complement levels
4. Immune complex deposition
5. Numerous other autoantibodies

Front 26

What is Systemic Lupus Erythematosus (SLE)

Back 26

autoimmune disease that involves chronic, systemic inflammation. Multiple organ systems are usually involved and the disease affects females more than males by a 4:1 ratio. The disease may have alternating remissions and exacerbations, and can be fairly mild or can result in death.
Major clinical presentations of SLE include arthritis, skin rash, pericarditis, nephritis, coronary disease, CNS complications, and vasculitis.

Front 27

what is a hallmark characteristic of Systemic Lupus Erythematosus (SLE)

Back 27

butterfly rash on face

Front 28

please describe the general features of RHEUMATOID ARTHRITIS (RA)

Back 28

Rheumatoid arthritis is a chronic, recurrent, systemic inflammatory disease that primarily involves the joints. RA affects females more than males by a 3:1 margin. RA characteristically begins in the small joints of the wrists and hands and progresses in centripetal and symmetric fashion. The cause is unknown.

Front 29

Major immunologic features of Rheumatoid Arthritis (5)

Back 29

1. Rheumatoid Factor
2. Immune Complexes
3. Inflammation within the joints
4. Joint deformity and destruction
5. Rheumatoid nodules

Front 30

TNF alpha and IL-1 cytokines tend to play a role in the pathogenesis of what disease?

Back 30

Rheumatoid Arthritis

Front 31

Please describe what happens in insulin-dependent diabetes mellitus (IDDM)

Back 31

Autoantibodies are produced that react with the beta cells of the pancreatic islets of Langerhans. This results in diminished insulin production.

Front 32

A 9-year-old boy was seen by his PCP with approximately 3-week history of excessive thirst, polyuria, and a weight loss of 3 kg. According to his mother he seemed lethargic during the day, and slept excessively at night. He reported some blurred vision . . .
what does he have?

Back 32

insulin-dependent diabetes mellitus (IDDM)

Front 33

What is killing the Beta cells in the Islets of Langerhans in IDDM?

Back 33

Cytokine-producing cells probably induce inflammatory reactions which result in beta cell destruction. Autoantibody, complement, and CD8+ cytotoxic T cells have all been demonstrated in diseased pancreas

Front 34

THYROIDITIS (Hashimoto's thyroiditis) 4

Back 34

• Inflammatory disorder of the thyroid, of unknown etiology; ~85% in females
• Cellular infiltration of the thyroid (plasma cells, T cells and macrophages)
• High titer autoantibodies produced
• Patient will have enlarged thyroid (goiter) and abnormal thyroid function (hypothyroid)

Front 35

A 13-year-old female patient presents with vague abdominal discomfort, malaise, and occasional non-bloody diarrhea over a 5-month period. Her mother stated there was weight loss and anorexia. Examination revealed hyper pigmentation, even on buccal mucosa and gingiva. Lab tests revealed low cortisol levels and antibodies to the adrenal cortex. The most likely diagnosis is

Back 35

Addison's disease

Front 36

What disease will target enzymes involved in steroid synthesis?

Back 36

Addison's Disease

Front 37

Patient presentation for Addison's disease?

Back 37

Patients show hypotension, anorexia, malaise and hyperpigmentation

Front 38

Addison's disease is auto-antibody against what?

Back 38

adrenal cells

Target auto-antigens may be enzymes involved in steroid synthesis
Patients show hypotension, anorexia, malaise and hyperpigmentation
Often associated with other endocrine

Front 39

What is a Polyglandular Autoimmune Syndrome?

Back 39

when you have one endocrine disease, it is normally accompanied with another one; Addison's disease is normally seen with another endocrine disease

Front 40

Graves’ Disease (Thyrotoxicosis)

Back 40

Autoantibody against the TSH receptor (stimulates it)


Signs of hyperthyroidism

Front 41

Bulging of the eyes (proptosis) is an indication of what disease?

Back 41

Graves' Disease

Front 42

Your 32-year-old female patient presents with a prominent erythematous rash over her nose and cheeks. She says the rash became noticeable during her vacation in Florida. She also complains of intermittent fever, fatigue, and some pain and stiffness in her wrists. You suspect systemic lupus erythematosus (SLE). Which of the following laboratory tests is most significant for the diagnosis of this disease?

A. Elevated Sed rate and CRP
B. Hypergammaglobulinemia
C. Auto-antibody against double-stranded DNA
D. Positive rheumatoid factor
E. Collagen-specific auto-reactive T cells

Back 42

C. Auto-antibody against double-stranded DNA

Front 43

Goodpasture’s Syndrome, please give the triad seen

Back 43

triad of pulmonary hemorrhage, glomerulonephritis, and circulating antibody to basement membranes.

affects primarily young males

Front 44

what anti- antibodies are seen in Goodpasture's Syndrome?

Back 44

anti-GBM antibodies

Front 45

What is Inflammatory Bowel Disease and the 2 types?

Back 45

IBD are chronic idiopathic inflammatory bowel diseases believed to be due, in part, to disregulation of the immune system

1. Ulcerative colitis (UC) and 2. Crohn's disease (CD)

Front 46

Ulcerative colitis (UC) presents how?

Back 46

bloody diarrhea, tenesmus, fever, weakness

Front 47

how does Chrohn's disease present?

Back 47

abdominal pain, diarrhea, malabsorption

Front 48

Where does Crohn's Disease affect a patient?

Back 48

can involve any part of the bowel wall; transmural inflammation

Front 49

where does Ulcerative colitis (UC) affect a patient

Back 49

limited to the colon; superficial layers of wall

Front 50

What are skip lesions? what are they seen in

Back 50

in Crohn's Disease you may see patches that are affected, then parts that aren't, then parts that are again, and so on

Front 51

What are some of the causes of immune hemolytic anemias? 3

Back 51

1) Alloantibody (for example, from incompatible blood transfusion)
2) Autoantibody - arising from no known cause or in conjunction with other disease
3) Drug-induced

Front 52

What antibodies can be involved in hemolytic anemia?

Back 52

IgM , IgG1 or IgG3 antibodies, due to their superior ability to activate complement

Front 53

What causes myasthenia gravis?

Back 53

auto-reactive antibodies are directed against the acetylcholine receptors at neuromuscular junctions

Complement activated or ligand-receptor internalization lead to reduction in post-synaptic acetylcholine receptors and post-synaptic membrane destruction. The result is muscle weakness.

Front 54

What are the general characteristics of inflammatory vasculitides

Back 54

This is a group of inflammatory disorders with a common target organ, the vascular system. The common pathological finding is inflammation of the blood vessels

Front 55

What diseases can present with vasculitis?

Back 55

SLE, Rheumatoid arthritis, infections

Front 56

pt. presents with blood supply to organs/tissue impaired
rash, fever, fatigue, abnormal or loss of sensations, joint pain, dark urine

what do they have?

Back 56

Vasculitis

Front 57

Main feature of Multiple Sclerosis

Back 57

MS is a chronic debilitating disease that features inflammatory demyelination of the CNS, resulting in progressive motor weakness, parethesias, visual impairment, ataxia . . .

Front 58

Please list 4 immunologic features of MS

Back 58

Inflammatory demyelination via macrophages & CTL
Oligoclonal immunoglobulin bands in CSF
HLA antigen associations
Altered immunoregulatory T cell populations