Deck 8: Autoimmune Diseases and Immunodeficiency Disorders

Question 1

A 19- year- old college student was evaluated for a red rash on her face after a day at the beach. The student stated that the rash looked different from sunburn and that since she had been feeling generally unwell lately that she decided to have it checked out. Laboratory results were as follows. What is the most reasonable diagnosis and what follow- up testing is most appropriate? Complete blood count (CBC)-slightly low red blood cells, platelets, and white blood cells Urinalysis-2+ protein
Renal function tests (BUN and creatinine)-elevated Liver function tests-within normal limits Electrolytes and glucose-within normal limits
Antinuclear antibody (ANA)-positive, homogeneous pattern, titer 1:256 Total complement-decreased
Complement C3-decreased Complement C4-decreased

A)Systemic lupus erythematosus/antiglomerular basement membrane antibody
B)Systemic lupus erythematosus/anti- DS DNA
C)Autoimmune hepatitis/antismooth muscle antibody
D)Goodpasture's syndrome/rheumatoid factor
E)Goodpasture's syndrome/antiglomerular basement membrane antibody

Answer 1

Systemic lupus erythematosus/anti- DS DNA

Question 2

Which condition is associated with progressive thickening of the skin, vascular lesions causing organ damage, and a positive nucleolar ANA?

A)Primary biliary cirrhosis
B)Antiphospholipid syndrome
C)Sjogren's syndrome
D)Dermatomyositis
E)Progressive systemic sclerosis

Answer 2

Progressive systemic sclerosis

Question 3

The strongest evidence suggests that type I diabetes mellitus is an autoimmune disease caused by

A)a defect in T suppressor cells and broken peripheral tolerance.
B)a pancreatic infection with bacteria that produce heat shock proteins similar to those in human tissue.
C)molecular mimicry resulting from a virus infection.
D)a forbidden B cell clone that was not deleted during fetal development.

Answer 3

molecular mimicry resulting from a virus infection.

Question 4

Some of the body's cells get very low levels of lymphocyte traffic and the body's lymphocytes rarely encounter the unique antigens in those body sites. Thus, if lymphocytes are suddenly exposed to those antigens, they may initiate an immune response against those cells. Which theory of autoimmunity is this?

A)Immunologic deficiency theory
B)Forbidden clone theory
C)Sequestered antigen theory
D)Altered antigen theory
E)Broken tolerance theory

Question 5

Which of the following is(are)highly specific for systemic lupus erythematosus (SLE)?

A)Speckled ANA and anti- Smith (Sm)antibody
B)Positive kinetoplast staining in Crithidia luciliae
C)Rim ANA
D)Homogeneous ANA
E)All of the above

Question 6

Which condition is associated with a positive speckled ANA, dry mouth, and dry eyes?

A)Dermatomyositis
B)Antiphospholipid syndrome
C)Progressive systemic sclerosis
D)Primary biliary cirrhosis
E)Sjogren's syndrome

Question 7

Immunodeficiencies involving reduced immunoglobulins and complement components lead to increased infections with

A)encapsulated bacteria.
B)fungi.
C)protozoa.
D)intracellular bacteria.
E)viruses.

Question 8

Which of the general statement(s)about autoimmunity is (are)TRUE?

A)The incidence of autoimmunity increases with age.
B)There are strong genetic links of some autoimmune diseases.
C)Women are at greater risk for autoimmune disease than men.
D)All of the above.

Question 9

A 58- year- old female had three subcutaneous nodules in her finger joints. She complained of extreme morning stiffness and pain in her joints that did improve during the course of the day. On physical exam she had a low grade fever, but was otherwise normal. Fluid withdrawn from the joint was cloudy and white. Immediate Gram stain of the fluid revealed mostly neutrophils but no organisms. Bacterial culture of the fluid was negative for organism growth. Which test below is most appropriate to do next?

A)Viral culture
B)C- reactive protein (CRP)
C)Rheumatoid factor (RF)
D)Erythrocyte sedimentation rate (ESR)
E)Antinuclear antibody (ANA)

Question 10

Joint damage in rheumatoid arthritis is primarily due to

A)complement and enzymes released by neutrophils during phagocytosis.
B)natural killer cell direct cell to cell killing.
C)T cytotoxic cell direct cell to cell killing.
D)All of the above.

Question 11

A 45- year- old female was evaluated for fatigue, lethargy, and a 25- pound weight gain since her las physical. Her face was slightly puffy, and her neck was slightly swollen. Laboratory results were as What is the most likely diagnosis? Complete blood count (CBC)-within normal limits Urinalysis-within normal limits
Renal function tests (BUN and creatinine)-within normal limits Electrolytes and glucose-within normal limits
Antinuclear antibody (ANA)-negative
Antithyroid peroxidase antibody (anti- TPO)-negative Antithyroglobulin antibody (anti- TG)-positive
TSH- receptor antibody (TRAb)-negative Total thyroxine hormone (T4)-low Thyroid stimulating hormone (TSH)-high

A)Hashimoto's thyroiditis
B)Systemic lupus erythematosus (SLE)
C)Goodpasture's syndrome
D)Type II diabetes mellitus
E)Graves' disease

Question 12

Anemia due to antibody to intrinsic factor would be most associated with which disease?

A)Idiopathic thrombocytopenia purpura
B)Pernicious anemia
C)Paroxysmal nocturnal hemoglobinuria
D)Autoimmune hemolytic anemia
E)Systemic lupus erythematosus

Question 13

A five- year- old child presents with repeated sinus infections due to Hemophilus influenzae and Streptococcus pneumoniae. On physical exam, the child appeared to have scoliosis and was not well developed for his age. He also appeared to have an inflammatory skin condition. Laboratory results as follows. What is the most likely diagnosis? Complete blood count-red cells, white cells and platelets within normal limits
WBC differential-50% neutrophils, 30% lymphocytes, 15% eosinophils, 5% monocytes Serum protein electrophoresis-within normal limits
Urinalysis-within normal limits serum IgG-within normal limits serum IgA-within normal limits serum IgM-within normal limits
Serum IgE -100 times the upper limit of normal

A)Severe combined immunodeficiency disorder
B)Chronic granulomatous disease
C)DiGeorge syndrome
D)Bruton's agammaglobulinemia
E)Job's syndrome

Question 14

A one- month- old child was evaluated for repeated respiratory infections, diarrhea, and failure to th On physical exam, it appeared that the child was missing her tonsils. Laboratory results were as foll What is the most likely diagnosis? White blood count 5302/uL
White blood cell differential 90% neutrophils, 5% monocytes, 2% eosinophils, 3% lymphocytes Serum protein electrophoresis-profoundly low gamma globulin

A)DiGeorge syndrome
B)Bruton's agammaglobulinemia
C)Chronic granulomatous disease
D)Job's syndrome
E)Severe combined immunodeficiency disorder

Question 15

When fighting an infectious agent, the immune system may make antibodies to that agent that cross- react with similar self- antigens. This mechanism of autoimmunity is called

A)epitope spreading.
B)superantigen induction.
C)molecular mimicry.
D)nonspecificity.

Question 16

Each immunodeficiency syndrome below is paired correctly with its underlying defect except

A)CD3 complex deficiency/no CD3 complex and therefore no T cells.
B)X- linked hyper- IgM/defect in T helper cell CD154 which is required to stimulate B cell CD40 for immunoglobulin class switching.
C)bare lymphocyte syndrome/no CD4 and CD8 complex and therefore no T cells.
D)Wiscott- Aldrich syndrome/defect in production of Wiscott- Aldrich protein necessary for lymphocytes to change cytoskeletal structure and respond to stimuli.
E)Bruton's X linked agammaglobulinemia/deficient B cell tyrosine kinase, arrested B cell maturation, and no circulating mature B cells.

Question 17

A 25- year- old female was evaluated for episodes of numbness and difficulty in moving her extremi She is currently asymptomatic. Laboratory results were as follows. What is the most likely diagnosis Complete blood count (CBC)-within normal limits Urinalysis-within normal limits
Renal function tests (BUN and creatinine)-within normal limits Electrolytes and glucose-within normal limits
Antinuclear antibody (ANA)-negative Antiacetylcholine receptor antibody-negative
Total protein, total IgG and myelin basic protein in cerebrospinal fluid (CSF)-elevated High resolution protein electrophoresis of serum-polyclonal gamma region
High resolution protein electrophoresis of CSF-oligoclonal gamma region

A)Myasthenia gravis (MG)
B)Multiple myeloma
C)Multiple sclerosis (MS)
D)Systemic lupus erythematosus (SLE)

Question 18

A 37- year- old Hispanic man who works in a chemical factory was seen because he was coughing u blood and experiencing chest pain. Laboratory results were as follows. What is the most likely diagn Complete blood count (CBC)-slight normocytic anemia; platelets and white blood cells within nor limits
Urinalysis-3+ protein, 1+ blood
Renal function tests (BUN and creatinine)-elevated Liver function tests-within normal limits Electrolytes and glucose-within normal limits Antinuclear antibody (ANA)-negative Antimitochondrial antibody-negative
Antismooth muscle antibody-negative
Antiglomerular basement membrane antibody-positive

A)Chronic active hepatitis
B)Systemic lupus erythematosus (SLE)
C)Idiopathic thromobocytopenia purpura (ITP)
D)Primary biliary cirrhosis
E)Goodpasture's syndrome

Question 19

A jaundiced 35- year- old white prostitute was evaluated for fatigue. Laboratory results were as foll What is the most likely diagnosis? Complete blood count (CBC)-within normal limits Urinalysis-2+ bilirubin
Renal function tests (BUN and creatinine)-within normal limits Liver function tests-liver enzymes and bilirubin elevated Electrolytes and glucose-within normal limits
Antinuclear antibody (ANA)-positive, speckled pattern Antimitochondrial antibody-negative
Antismooth muscle antibody-positive
Antineutrophil cytoplasmic antibody (ANCA)-negative Hepatitis A virus antibody-negative
Hepatitis B surface antigen-negative Antibody to hepatitis B core antigen-negative Antibody to hepatitis C antigen-negative Anti- HIV-negative

A)Primary sclerosing cholangitis
B)Viral hepatitis
C)Systemic lupus erythematosus (SLE)
D)Autoimmune hepatitis
E)Primary biliary cirrhosis

Question 20

When the thymus is congenitally missing or extremely reduced in size this is called

A)Job's syndrome.
B)chronic granulomatous disease.
C)Bruton's agammaglobulinemia.
D)severe combined immunodeficiency disorder.
E)DiGeorge syndrome.

Question 21

Cold autoimmune hemolytic anemias are usually due to autoantibodies directed against a high frequency Rh antigen on the patient's cells.

Question 22

What neutrophil function is defective in leukocyte adhesion defect?

A)Digestion
B)Opsonization
C)Phagocytosis
D)Diapedesis
E)Chemotaxis

Question 23

Patients who present with long bleeding times, easy bruising, and purpura should be evaluated for antibodies.

Question 24

Plasma that contains antiphospholipids has prolonged clotting times in test tube assays. However, many patients with these antibodies experience an increased tendency to form blood clots.

Question 25

Which of the following indicate that an HIV positive person has progressed into full blown AIDS?

A)Kaposi's sarcoma
B)CD4 count below 200 cells/mm3
C)Skin anergy to recall antigens
D)Pneumocystis carnii pneumonia
E)All of the above

Question 26

Autoantibodies are always damaging to host tissue.

Question 27

Systemic lupus erythematosus (SLE)patients who have a false positive syphilis test probably have circulating anticardiolipin antibody.

Question 28

All of the following are TRUE about the human immunodeficiency virus except

A)HIV integrates its own viral DNA into the host DNA.
B)HIV contains an enzyme the humans do not possess, reverse transcriptase.
C)HIV preferentially infects human cells expressing the CD4 marker.
D)HIV is a double- stranded DNA retrovirus.
E)HIV is transmitted through blood and body fluids, so major forms of transmission are sexual contact and exposure to blood (transfusion, dirty needles, etc.).

Question 29

The factor that inhibits complement- mediated lysis of red blood cells by dissociating C3 and C5 convertases is _. Deficiency of this factor causes .

A)CD59/paroxysmal nocturnal hemoglobinuria (PNH).
B)decay accelerating factor (DAF)/paroxysmal nocturnal hemoglobinuria (PNH).
C)C4 binding protein/angioedema.
D)C1 esterase inhibitor/angioedema.
E)C3 nephritic factor/partial lipodystrophy.

Question 30

The autoantibody most characteristic of Graves' disease is .

Question 31

A clinical lab scientist observing the staining in an antinuclear antibody (ANA)test noted that the nuclei of interphase cells appeared to have dozens of dots scattered evenly over the entire nucleus. Chromosomes at metaphase did not show any staining. This pattern should be reported as homogeneous.

Question 32

AIDS is uniformly fatal because the human immune system responds so poorly to the HIV virus.

Question 33

When drugs or viruses change self- antigens such that the immune system recognizes them as foreign, these altered self- antigens are called _.

Question 34

An IgM antibody directed at the Fc portion of IgG is called .

Question 35

Deficiencies in complement components 5 through 9 are particularly associated with

Question 36

Individuals who have had their spleens removed may be immunodeficient.

Question 37

Patients with active autoimmune diseases often have high blood C- reactive protein levels (CRP)and increased erythrocyte sedimentation rates (ESR).

Question 38

A three- year- old child presents with repeated staphylococcal skin infections that take a very long time to resolve. The total white blood cell count is within normal limits and counts of all white blood cell types are within normal limits. All immunoglobulin and complement levels are within normal limits. A nitroblue tetrazolium (NBT)test is performed, and at the end of incubation, all neutrophils appear yellow. What is the best interpretation of these data?

A)The child may have chronic granulomatous disease (CGD).
B)All tests done to date are within normal limits and additional testing should be done.
C)The child may have cyclic neutropenia.
D)The child may have leukocyte adhesion defect (LAD).
E)The child may have Job's syndrome.

Question 39

When kidney biopsies from patients with Goodpasture's syndrome are stained with fluorescent antihuman globulin, bright "beads" of fluorescence can be seen scattered randomly throughout the glomeruli.

Question 40

Rheumatoid arthritis is a progressive, asymmetrical, nondeforming arthritis that begins in the small joints (such as fingers)and progresses to the larger joints (such as hips).

Question 41

Explain the autoimmune mechanism of myasthenia gravis (MG).

Question 42

Describe how the lymphocyte development in the thymus minimizes T cells capable of inducing autoimmunity while maintaining T cells capable of recognizing foreign antigens.

Question 43

Explain how hematopoietic malignancies cause immunosuppression and how many treatments for malignancies also cause immunosuppression.

Question 44

Discuss how circulating immune complexes are formed in systemic lupus erythematosus (SLE), where they deposit, how tissue is destroyed, and how this relates to the cluster of symptoms associated with SLE.

Question 45

There are many defects that result in severe combined immunodeficiency disorder. List four and briefly define them.