4466_Ch15_233-262 30/08/16 5:06 PM Page 261
Chapter 15
261
Autoimmunity
CASE STUDIES
1. A 25-year-old female consulted her physician because
she had been experiencing symptoms of weight loss,
joint pain in the hands, and extreme fatigue. Her laboratory results were as follows: RF rapid slide test positive
at 1:10; ANA rapid slide test positive at 1:40; RBC 3.5 ×
1012 per L (normal is 4.1 to 5.1 × 1012 per L); WBC
count 5.8 × 109 per L (normal is 4.5 to 11 × 109 per L).
2. A 40-year-old female went to her doctor because she was
feeling tired all the time. She had gained about 10 pounds
in the last few months and exhibited some facial puffiness. Her thyroid gland was enlarged and rubbery. Laboratory results indicated a normal RBC and WBC count,
but her FT4 level was decreased and an assay for antithyroglobulin antibody was positive.
Questions
a. What is a possible explanation for positive results on
both the RF test and the ANA test?
b. What is the most likely cause of the decreased RBC
count?
c. What further testing would help the physician distinguish between RA and SLE?
Questions
a. What condition do these results likely indicate?
b. What effect do antithyroglobulin antibodies have on
thyroid function?
c. How can this condition be differentiated from Graves
disease?
REVIEW QUESTIONS
1. All of the following may contribute to autoimmunity
except
a. clonal deletion of self-reactive T cells.
b. molecular mimicry.
c. increased expression of class II MHC antigens.
d. polyclonal activation of B cells.
5. A peripheral pattern of staining of the nucleus on IIF is
caused by which of the following antibodies?
a. Anti-Sm antibody
b. Anti-SSA/Ro antibody
c. Centromere antibody
d. Anti-dsDNA
2. Which of the following would be considered an
organ-specific autoimmune disease?
a. SLE
b. RA
c. GPA
d. Hashimoto’s thyroiditis
6. Which of the following would be considered a significant finding in Graves disease?
a. Increased TSH levels
b. Antibody to TSHR
c. Decreased T3 and T4
d. Antithyroglobulin antibody
3. SLE can be distinguished from RA on the basis
of which of the following?
a. Joint pain
b. Presence of antinuclear antibodies
c. Immune complex formation with activation
of complement
d. Presence of anti-dsDNA antibodies
7. Destruction of the myelin sheath of axons caused by the
presence of antibody is characteristic of which disease?
a. MS
b. MG
c. Graves disease
d. Goodpasture’s syndrome
4. Which of the following would support a diagnosis
of drug-induced lupus?
a. Antihistone antibodies
b. Antibodies to Smith antigen
c. Presence of RF
d. Antibodies to SS-A and SS-B antigens
8. Blood was drawn from a 25-year-old woman with
suspected SLE. A FANA screen was performed and
a speckled pattern resulted. Which of the following
actions should be taken next?
a. Report out as diagnostic for SLE
b. Report out as drug-induced lupus
c. Perform an assay for specific ANAs
d. Repeat the test
4466_Ch15_233-262 30/08/16 5:06 PM Page 262
262
SECTION 3 Immune Disorders
9. Which of the following is a mechanism used to
achieve peripheral tolerance?
a. Negative selection of autoreactive T cells in the
thymus
b. Apoptosis of autoreactive B cells in the bone marrow
c. Editing of B-cell receptors that weakly recognize
self-antigens in the bone marrow
d. Lack of a costimulatory signal to autoreactive
T cells in the lymph nodes
10. Epitope spreading refers to
a. post-translational modifications to self-antigens.
b. modifications in gene expression that are not
caused by changes in DNA sequence.
c. expansion of the immune response to unrelated
antigens.
d. cross-reaction of the immune response to a
pathogen with a similar self-antigen.
11. Anti-CCP (cyclic citrullinated proteins) is specifically
associated with which autoimmune disease?
a. RA
b. MG
c. Autoimmune hepatitis
d. Goodpasture’s syndrome
12. Which autoantibodies are strongly associated with granulomatosis with polyangiitis (Wegener’s granulomatosis)?
a. ANA
b. ANCA
c. AMA
d. SMA
13. A technologist performs an IIF test for ANCAs and
observes that there is an intense fluorescent staining
of the nuclear lobes of the neutrophils. How can this
type of staining be differentiated from a peripheral
ANA pattern?
a. Perform the test on formalin-fixed leukocytes
b. Perform IIF with HEp-2 cells
c. Perform an ELISA for ANCAs
d. All of the above
14. A 20-year-old woman made an appointment to see her
physician because she was experiencing intermittent
diarrhea. Laboratory testing revealed that she also had
an iron deficiency anemia. To determine if the patient
has celiac disease, her doctor should order which of
the following laboratory tests?
a. Anti-tTG
b. Antigliadin
c. Antigluten
d. All of the above
15. Antimitochondrial antibodies are strongly associated
with which disease?
a. Autoimmune hepatitis
b. Celiac disease
c. Primary biliary cirrhosis
d. Goodpasture’s syndrome
4466_Ch16_263-277 30/08/16 5:05 PM Page 276
276
SECTION 3 Immune Disorders
REVIEW QUESTIONS
1. Which of the following responses is the type of allograft
rejection associated with vascular and parenchymal
injury with lymphocyte infiltrates?
a. Hyperacute rejection
b. Acute cellular rejection
c. Acute humoral rejection
d. Chronic rejection
2. Antigen receptors on T lymphocytes bind HLA class II
+ peptide complexes with the help of which accessory
molecule?
a. CD2
b. CD3
c. CD4
d. CD8
3. Patients who have received the following types of
grafts are at risk for graft-versus-host disease (GVHD)
except for recipients of
a. bone marrow transplants.
b. lung transplants.
c. liver transplants.
d. irradiated leukocytes.
4. Which of the following properties are not exhibited
by HLA molecules?
a. They belong to the immunoglobulin superfamily.
b. They are heterodimeric.
c. They are integral cell membrane glycoproteins.
d. They are monomorphic.
5. Kidney allograft loss from intravascular thrombosis
without cellular infiltration 5 days post-transplant
may indicate which primary rejection mechanism?
a. Hyperacute rejection
b. Accelerated humoral rejection
c. Acute humoral rejection
d. Acute cellular rejection
6. Which reagents would be used in a direct (forward)
donor–recipient crossmatch test?
a. Donor serum and recipient lymphocytes + rabbit
serum complement
b. Recipient serum and donor lymphocytes + rabbit
serum complement
c. Donor stimulator cells + recipient responder cells +
complete culture medium
d. Recipient stimulator cells + donor responder cells +
complete culture medium
7. The indirect allorecognition pathway involves which
one of the following mechanisms?
a. Processed peptides from polymorphic donor
proteins restricted by recipient HLA class II
molecules
b. Processed peptides from polymorphic recipient
proteins restricted by donor HLA class I molecules
c. Intact polymorphic donor protein molecules
recognized by recipient HLA class I molecules
d. Intact polymorphic donor protein molecules
recognized by recipient HLA class II molecules
8. Which immunosuppressive agent selectively
inhibits IL-2 receptor-mediated activation of T cells
and causes clearance of activated T cells from the
circulation?
a. Mycophenolate mofetil
b. Cyclosporine mofetil
c. Corticosteroids
d. Daclizumab
9. Phenotyping for HLA class II antigens requires
B lymphocytes because
a. B lymphocytes express HLA class II antigens.
b. B lymphocytes do not express HLA class I
antigens.
c. B lymphocytes are exquisitely sensitive to
complement-mediated lysis.
d. B lymphocytes represent the majority of lymphocytes
in the peripheral blood.
10. A renal transplant candidate was crossmatched with a
donor that was mismatched for only the HLA-B35
antigen. The candidate was known to have an antibody specific for HLA-B35. Which of the following
combinations of T- and B-cell flow cytometric
crossmatch results would be expected?
a. T cell negative, B cell negative
b. T cell positive, B cell positive
c. T cell negative, B cell positive
d. T cell positive, B cell negative
11. Which of the following HLA alleles differs from
A*02:01:02 by a synonymous nucleotide
substitution?
a. A*01:01:01:01
b. A*02:01:03
c. A*02:02
d. A*02:03:01
4466_Ch16_263-277 30/08/16 5:05 PM Page 277
Chapter 16
Transplantation Immunology
277
12. Which one of the following donors would be expected
to elicit a positive mixed lymphocyte response in lymphocytes from a patient who has the HLA-DRB1*01:01,
01:03 alleles?
a. DRB1*01:01, 01:03
b. DRB1*01:01, 01:01
c. DRB1*01:03, 01:03
d. DRB1*01:01, 01:05
14. Which of the following HLA antigens would be expected to elicit an HLA antibody response in a kidney
transplant recipient with the following HLA type:
HLA-A*01,03; B*07,14; C*01,04N; DRB1*16,07?
a. HLA-A*01
b. HLA-B*14
c. HLA-C*04
d. HLA-DRB1*16
13. Which of the following donors would be the most
appropriate, based on ABO compatibility, for a renal
transplant candidate with the ABO type = O?
a. O
b. A
c. B
d. AB
15. Suppose a 30-year-old man was found to be a suitable
donor for a kidney transplant to his younger sister.
This transplant would be an example of a(an)
a. autograft.
b. allograft.
c. isograft.
d. xenograft.