Deck 16: Disruption of Healthy Tissue by the Adaptive Immune Response

Question 1

All of the following are true regarding thyroglobulin except _____.

A)its tyrosine residues are iodinated and cross-linked
B)it is broken down to produce thyroid hormones
C)it is stored in follicles of the thyroid
D)it signals the pituitary gland to stop releasing thyroid-stimulating hormone
E)it is synthesized initially as a glycoprotein by thyroid epithelial cells.

Answer 1

D

Question 2

Explain why splenectomy is sometimes carried out in patients with persistent type II autoimmune diseases that affect leukocytes.

Answer 2

When autoantibodies directed toward leukocyte surface antigens bind to the cell surface,complement fixation and deposition of C3b occurs.The membrane-attack complex usually does not form on leukocytes because of complement regulatory proteins.Their elimination from the circulation,however,occurs by a different mechanism involving splenic macrophages that bear FcR and CR1,receptors for antibodies and C3b,respectively.The macrophages carry out phagocyte-mediated clearance of these leukocytes,leading to a deficiency of these cells in the circulation.Removal of the spleen spares the elimination of these leukocytes.The presence of antibody and C3b on their cell surface does not affect their ability to functional normally.

Question 3

Which of the following is an example of a type III autoimmune response? (Select all that apply.)

A)mixed essential cryoglobulinemia
B)acute thrombocytopenia purpura
C)systemic lupus erythematosus
D)rheumatoid arthritis
E)insulin-resistant diabetes.

Answer 3

A,C

Question 4

The reason why babies born to mothers with Graves' disease suffer passively from the disease for only a short while after birth is that _____.

A)very little IgM is transported across the placenta
B)only antibodies,and not the B cells making the autoantibodies,cross the placenta
C)the newborn's regulatory T cells suppress autoantibody production
D)the newborn's thyroid gland develops resistance to the effects of maternal autoantibodies
E)thyroglobulin synthesis does not commence until months after birth.

Question 5

A(n)_____ binds to the antigen-binding site of another antibody.

A)cryptic epitope
B)anti-idiotypic antibody
C)molecular mimic
D)receptor antagonist
E)autoantibody.

Question 6

Autoimmune diseases,which are classified on the basis of the effector mechanism that causes the symptoms,include all of the following types of hypersensitivity reaction except _____.

A)type I
B)type II
C)type III
D)type IV.

Question 7

Which of the following is associated with antagonistic autoantibodies against cell-surface receptors or adhesion molecules? (Select all that apply.)

A)myasthenia gravis
B)rheumatoid arthritis
C)insulin-resistant diabetes
D)Graves' disease
E)pemphigus vulgaris.

Question 8

Which of the following describes myasthenia gravis?

A)Ectopic lymphoid tissue forms and impairs endocrine function.
B)Disruption of adhesion molecules of cellular junctions.
C)Autoimmune response to proteins of anterior chamber of the eye.
D)Chronic inflammation of the gut mucosa.
E)The neuromuscular junction is compromised.

Question 9

It is believed that the allotype DRB1*_____ may confer protection against rheumatoid arthritis because it contains _____ amino acid residues at positions 70 and 71 that bind to different subsets of peptides compared with the allotypes that confer susceptibility to this disease.

A)04:01; acidic
B)04:02; acidic
C)04:04; basic
D)04:05; basic e 04:08; acidic.

Question 10

Which of the following is the cause of red blood cell deficiency in autoimmune hemolytic anemia? (Select all that apply.)

A)inability of red blood cells to develop normally in the bone marrow
B)loss of red blood cells due to widespread pinpoint hemorrhages
C)hemolysis by assembly of membrane-attack complexes
D)phagocyte-mediated clearance in the spleen
E)rapid turnover of red blood cells due to CD8 T-cell killing.

Question 11

With the exception of ______,these autoimmune diseases are more prevalent in women than in men.

A)rheumatoid arthritis
B)multiple sclerosis
C)ankylosing spondylitis
D)Sjögren's syndrome
E)Graves' disease.

Question 12

Which of the following is not a characteristic of Graves' disease?

A)weight loss
B)enlarged thyroid gland
C)elevated thyroid-stimulating hormone
D)heat intolerance
E)overproduction of T₃ and T₄.

Question 13

All of the following autoimmune diseases are correctly matched with their HLA disease associations except _____.

A)HLA-B27: ankylosing spondylitis
B)HLA-DQ2: type 1 diabetes in Africans and Asians
C)HLA-B35: birdshot retinopathy
D)HLA-DR4: rheumatoid arthritis
E)HLA-DQ6: narcolepsy.

Question 14

Which of the following is an example of a type II autoimmune response? (Select all that apply.)

A)subacute bacterial endocarditis
B)Goodpasture's syndrome
C)multiple sclerosis
D)systemic lupus erythematosus
E)myasthenia gravis.

Question 15

Individuals who have two defective alleles of the AIRE gene _____.

A)exhibit symptoms of autoimmunity at a young age
B)are unable to activate regulatory T cells
C)exhibit decreased predisposition to autoimmune disease
D)are very effective at inducing anergy of circulating autoreactive B and T cells
E)are more likely to be women of African or Asian origin.

Question 16

autoantibodies of the IgG or IgM isotype were produced with specificity for components found on the surface of erythrocytes,which of the following would occur? (Select all that apply.)

A)formation of membrane-attack complex
B)immune-complex deposition in renal glomeruli
C)anemia
D)hypothyroidism
E)receptor-mediated phagoytosis via Fc receptors on phagocytes.

Question 17

Which of the following is an example of a type IV autoimmune response? (Select all that apply.)

A)pemphigus vulgaris
B)autoimmune thrombocytopenia purpura
C)subacute bacterial endocarditis
D)type 1 diabetes
E)multiple sclerosis.

Question 18

Which type of autoimmune disease is correctly matched with its cause?

A)type I: IgE-mediated
B)type II: effector T cells
C)type III: immune complex deposition in tissues
D)type IV: extracellular matrix-associated autoantigens
E)type V: cell-surface components.

Question 19

_____ is a highly variable type III autoimmune disease in which immune complexes form and may cause glomerulonephritis of the kidney,arthritis of the joints,and vasculitis of the face.

A)pemphigus vulgaris
B)systemic lupus erythematosus
C)rheumatoid arthritis
D)multiple sclerosis
E)Goodpasture's syndrome.

Question 21

Describe the three immunological mechanisms responsible for the destruction of red blood cells in autoimmune hemolytic anemia.

Question 22

Characterize two properties of endocrine glands that render them susceptible to autoimmune attack.

Question 23

Explain the relationship between HLA-DRB1*04,smoking,the expression of peptidyl arginine deaminase,and rheumatoid arthritis.

Question 24

Hashimoto's and Graves' diseases both impair normal functioning of the thyroid gland but do so using different immunopathological mechanisms.Compare and contrast these mechanisms.

Question 26

People who are heterozygous for HLA-DQ2 and HLA-DQ8 allotypes are at greater risk of developing type 1 diabetes than those who are homozygous for HLA-DQ2 or HLA-DQ8.
A.Explain the reason for this increased susceptibility.
B.Why is the above statement true mainly for people of northern European origin but not for some other ethnic groups?

Question 27

Which patients affected by Goodpasture's syndrome also succumb to pulmonary hemorrhage?
B.Explain the reason for this complication.

Question 28

Which of the following would be consistent with a diagnosis of Goodpasture's syndrome? (Select all that apply.)

A)pulmonary hemorrhage
B)joint inflammation
C)glomerulonephritis
D)anti-collagen IgG deposition in renal glomeruli
E)hyperglycemia.

Question 29

A recent therapy developed for the treatment of rheumatoid arthritis includes the use of _____ monoclonal antibodies that suppress the autoimmune response.(Select all that apply.)

A)anti-TNF-α
B)anti-C-reactive protein
C)anti-CD20
D)anti-rheumatoid factor
E)anti-CD3.

Question 30

All of the following are characteristics of tissue transglutaminase except ____.

A)generation of negatively charged peptides that bind well to the positively charged pockets of the DQ2 and DQ8 allotypes
B)conversion of glutamine to glutamate by deamination
C)upregulation during tissue inflammation
D)stimulation of IgG or IgA autoantibodies in celiac disease
E)predisposition to celiac disease if individual possesses particular polymorphic variants.

Question 31

You have isolated a subset of CD25⁺ CD4⁺ T cells from the blood that have T-cell receptors specific for a self antigen but do not proliferate when challenged with the antigen in vitro.What is the name given to these T cells,and what role are they thought to have in preventing autoimmunity?

Question 32

Thyroid-stimulating hormone is made in the _____ and induces the release of thyroid hormones after proteolytic processing of _____.

A)pituitary gland; thyroglobulin
B)hypothalamus; thyroxine
C)pancreas; thyroglobulin
D)pituitary gland; thyroid-stimulating hormone receptor
E)thyroid gland; thyroid peroxidase.

Question 33

Discuss why splenectomy is a viable treatment for chronic autoimmune diseases targeted at circulating neutrophils.

Question 34

Describe three types of unwanted and potentially harmful immune response.

Question 35

Chronic diseases in which the immune response is targeted toward autologous entities of one's body are known as _____.

A)hypersensitivity reactions
B)innate immune reactions
C)allergic reactions
D)autoimmune diseases
E)anergic reactions.

Question 36

What mechanism of self-tolerance is broken in the autoimmune syndrome APECED?
B.What is the underlying genetic defect in APECED? Explain why it leads to a reduction in self-tolerance.

Question 37

In the context of autoimmunity: (A)define molecular mimicry; and (B)provide an example.

Question 38

Which of the following are correctly matched? (Select all that apply.)

A)exocrine tissue: islets of Langerhans
B)type 2 diabetes: insulin-dependent diabetes mellitus
C)β cells of pancreas: insulin production
D)α cells of pancreas: somatostatin production
E)insulitis: lymphocyte infiltration in islets of Langerhans.

Question 39

_____ is the term used to describe how pathogen antigens resemble host antigens and can sometimes trigger autoimmune disease.

A)intramolecular epitope spreading
B)molecular mimicry
C)intermolecular epitope spreading
D)sympathetic senescence
E)linkage equilibrium.

Question 40

_____ autoantibodies enhance receptor function.

A)neutralizing
B)opsonizing
C)agonist
D)complement-fixing
E)antagonist.

Question 41

How do the treatments for Hashimoto's and Graves' diseases differ,and why?

Question 42

Bacterial infections are associated with which of the following autoimmune diseases? (Select all that apply.)

A)Reiter's syndrome
B)pemphigus vulgaris
C)reactive arthritis
D)rheumatic fever
E)myasthenia gravis.

Question 43

Rituximab,used in the treatment of rheumatoid arthritis,depletes _____ through a process involving the cross-linking of _____ on the surface of NK cells and the induction of antibody-dependent cell-mediated cytotoxicity.

A)NK cells; NKG2D
B)T cells: NKG2D
C)inflammatory cytokines; TNF-alpha
D)C-reactive protein; FcγRIII
E)B cells; FcγRIII.

Question 44

The process by which the human thymus gradually decays is known as _____.

A)apoptosis
B)senescence
C)involution
D)the hygiene hypothesis
E)self-tolerance.

Question 45

Graves' disease causes _____,whereas Hashimoto's disease causes _____.

A)hypothyroidism; hyperthyroidism
B)hyperthyroidism; hypothyroidism
C)hypoglycemia; hyperglycemia
D)hyperglycemia; hypoglycemia
E)glomerulonephtitis; systemic vasculitis.

Question 46

_____ is an example in which physical trauma provides access of lymphocytes to an otherwise immunologically privileged site.(Select all that apply.)

A)rheumatoid arthritis
B)multiple sclerosis
C)type 1 diabetes
D)myasthenia gravis
E)sympathetic ophthalmia.

Question 47

Describe two different ways in which infection with bacteria or viruses compromises T-cell tolerance,leading to the production of effector T cells specific for self antigens.

Question 48

The autoreactive CD4 T cells of elderly people with rheumatoid arthritis _____.(Select all that apply.)

A)express high levels of CD28
B)are predominantly anergic
C)express KIR2DS2
D)are highly susceptible to apoptosis in inflamed joints
E)produce IFN-γ.

Question 49

Which of the following autoimmune diseases affect the nervous system? (Select all that apply.)

A)myasthenia gravis
B)mixed essential cryoglobulinemia
C)Graves' disease
D)pemphigus vulgaris
E)multiple sclerosis.

Question 50

A(n)_____ is an epitope that is typically not accessible to the immune system but is revealed under inflammatory or infectious states.

A)cryptic epitope
B)molecular mimic
C)regulatory peptide
D)carrier
E)adjuvant.

Question 51

Explain the mechanism that gives rise to a broadening B-cell response during the course of systemic lupus erythematosus.

Question 52

Deficiency in the production of AIRE results in _____.(Select all that apply.)

A)normal expression of tissue-specific proteins in the bone marrow and thymus
B)incomplete negative selection of developing T cells
C)the development of autoimmune B-cell and T-cell responses against endocrine glands and other tissues
D)death in infancy
E)the development of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).

Question 53

What is ectopic lymphoid tissue?
B.Give four examples where this type of tissue forms in autoimmune disease.

Question 54

Autoantibody specificities are affected by HLA class II polymorphisms.In the case of systemic lupus erythematosus,indicate which of the following associations between HLA-class II and autoantigens have been observed in these patients.

A)HLA-DR3; nuclear ribonucleoprotein complex
B)HLA-DR5; small cytoplasmic ribonucleoprotein complex
C)HLA-DR2; double-stranded DNA
D)HLA-DR4; single-stranded RNA
E)HLA-DQ8; double-stranded RNA.

Question 55

The upregulation of _____ by IFN-γ can contribute to antigen-specific T-cell activation on thyroid epithelium.

A)CD4
B)CD8
C)HLA class I
D)HLA class II
E)CD28.

Question 56

Antagonistic autoantibodies made against the insulin receptor cause _____.(Select all that apply.)

A)type 1 diabetes
B)hypoglycemia
C)hyperglycemia
D)insulin-resistant diabetes
E)light-headedness.

Question 57

What is meant by the term 'epitope spreading'?
B.Name one autoimmune disease affecting the skin in which epitope spreading is involved,and explain how.

Question 58

Anders Anderson,was seen by his pediatrician at 24 months old after a recent bout of diarrhea and vomiting.He had lost his appetite and complained that his stomach hurt.Anders was in the 5% centile for weight,had slender limbs,wasted buttocks,and a protuberant abdomen.Jejunal biopsy revealed abnormal surface epithelium,and villous atrophy with hyperplasia of the crypts.Which of the following would be a likely clinical finding in this patient?
a.glomerulonephritis
b.urticarial rash
c.anti-gliadin IgA antibodies
d.chronic wheezing
e.low blood pressure.

Question 59

Seventeen-year-old Lisa Montague practiced piano for 3-4 hours each day while preparing for music college auditions.Some of her pieces required sustained arm-muscle activity and she began to find them hard to play,even though she had previously played them easily.When she also started to have difficulty swallowing and chewing,she told her mother,who took her to the emergency room,where the physician noticed drooping eyelids and limitation of ocular motility.An electromyogram detected impaired nerve-to-muscle transmission.Administration of pyridostigmine rapidly improved Lisa's symptoms.Which of the following blood-test results would be most consistent with her condition?
a.elevated rheumatoid factor
b.elevated anti-myelin basic protein antibodies
c.elevated anti-acetylcholine receptor antibodies
d.elevated anti-nuclear antibodies
e.elevated anti-Rh antibodies.
ANSWERS

Question 60

Amanda Chenoweth,21 years of age,returned from a summer job as a pianist on a cruise ship where she was exposed daily to excessive sun; she developed a rash on her cheeks.She complained that her finger joints were stiff and painful,which made it difficult to play the piano,and that her hips became painful after sitting at the piano for long periods.Her blood sample tested positive for anti-nuclear antibodies and had decreased serum C3 levels.A urine albumin test showed elevated protein levels.A course of prednisone (an anti-inflammatory steroid)in combination with naprosyn (a nonsteroidal anti-inflammatory agent)was begun and her condition improved rapidly.What is the most likely cause and clinical name of her condition?
a.deterioration of the central nervous system; multiple sclerosis
b.cartilage destruction by bone-cell enzymes; rheumatoid arthritis
c.immune complexes fixing complement in kidney,joints,and blood vessels; systemic lupus erythematosus
d.autoantibodies against acetylcholine receptor at the neuromuscular junction; myasthenia gravis
e.consumption of seafood to which she was allergic; acute systemic anaphylaxis.

Question 61

At 42 years old,Stephanie Goldstein developed occasional blurred and double vision,numbness and 'pins and needles' in her arms and legs (paresthesia),and bladder incontinence.After a month of these symptoms she went to her doctor,who sent her to the neurology specialist.An MRI scan revealed areas of demyelination in the central nervous system (CNS),and Stephanie was diagnosed with the autoimmune disease multiple sclerosis (MS).Which of the following best explains why some people are susceptible to the development of MS?
a.Negative selection of autoreactive T cells occurs during T-cell development.
b.Apoptosis of autoreactive B cells occurs in the bone marrow during B-cell development.
c.An inability to produce immunological tolerance toward CNS-derived constituents results in the generation of self-reactive lymphocytes.
d.An immunodeficiency inhibiting somatic recombination of immunoglobulins and T-cell receptors results in impaired lymphocyte development.
e.Regulatory T cells fail to activate autoreactive T cells in secondary lymphoid organs.