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CP Reviewer 2 > Immunology > Flashcards

Immunology Flashcards

1
Q

Immunoglobulin heavy chains: Domains.

A

One variable domain and 3-4 constant domains.

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2
Q

Paratope: Definition.

A

The part of the immunoglobulin molecular that binds the antigen.

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3
Q

Location of genes for immunoglobulin light chains and heavy chains.

A

Light chains: Chromosomes 2 (κ) and 22 (λ).

Heavy chains: Chromosome 14.

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4
Q

First step in rearrangement of immunoglobulin genes in B cells.

A

Rearrangement of genes for variable regions: V and J regions of light chains and V, D, and J regions of heavy chains.

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5
Q

Second step in rearrangement of immunoglobulin genes in B cells.

A

The rearranged sequences of the variable regions are joined to a gene sequence of a constant region of a light chain or heavy chain. The initial heavy-chain constant region is always μ.

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6
Q

Immunoglobulins on mature B cells.

A

IgM and IgD.

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7
Q

What happens when a helper T cell stimulates a mature B cell?

A

The B cell proliferates, and each progeny joins its prefabricated variable-gene sequences to a gene for a different heavy chain. This is isotope switching.

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8
Q

Idiotope: Definition.

A

The part of the (variable region of the) immunoglobulin molecule that can function as an epitope.

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9
Q

Which classes of immunoglobulin have subclasses?

A

IgG: 4 subclasses.

IgA: 2 subclasses.

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10
Q

Which classes of immunoglobulin can activate complement?

A

IgG1, IgG3, IgM: Classic pathway.

IgA: Alternate pathway.

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11
Q

Which class of immunoglobulin is the least abundant?

A

IgE.

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12
Q

Genes for the T-cell receptor:

A. Number.
B. Location.

A

A. Four genes: α, β, γ, δ.

B. Chromosome 7.

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13
Q

Types of T-cell receptors.

A

αβ: 95% of T lymphocytes.

γδ: Mostly in mucous membranes and skin.

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14
Q

NK cells:

A. Percentage of circulating lymphocytes.
B. TCR and immunoglobulin.

A

A. About 10%.

B. Not expressed; genes remain in germline state.

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15
Q

NK cells: Surface markers.

A

Positive: CD16, CD56, CD57.

Negative: CD3.

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16
Q

NK cells: Purpose of CD16.

A

As the FcγR, it binds opsonized cells, mediating antigen-dependent cytotoxicity.

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17
Q

NK cells: Functions.

A

Combating virus-infected cells and tumor cells.

Secreting IFNγ.

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18
Q

Antigen-presenting cells: Immunity-mediating antigens expressed on their surfaces.

A

All: MHC class II, CD68, lysozyme.

All but monocytes and macrophages: S100, CD1a.

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19
Q

Antigen-presenting cells: Secretion.

A

Interleukin 1.

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20
Q

Chemokine that attracts neutrophils.

A

Interleukin 8 (among others).

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21
Q

How basophils and mast cells come to release the contents of their granules.

A

They use their Fcε to bind IgE, the cross-linking of which promotes degranulation.

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22
Q

Cytokines that stimulate eosinophils.

A

IL-4 stimulates production of IgE.
IL-5 attracts eosinophils.

Both interleukins are secreted by Th2 cells.

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23
Q

Charcot-Leyden crystals:

A. Origin.
B. Function.

A

A. Degranulation of eosinophils.

B. They have lysophospholipase activity.

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24
Q

Complement: Final common pathway.

A

Formation of C5b6789, the membrane-attack complex.

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25
Q

Complement: Activators of the classic pathway.

A

Antigen-antibody complexes of IgG or IgM.

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26
Q

Complement: Classic pathway to the formation of the C3 convertase.

A

The Fc portion of the immunoglobulin molecule binds to C1q. Activated C1 catalyzes the formation of C4b2a.

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27
Q

Complement: Classic pathway from the formation of the C3 convertase.

A

C4b2a converts C3 to C3a and C3b. The latter joins the complex to make C4b2a3b, the C5 convertase, which yields C5b.

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28
Q

Complement: Alternative pathway to the formation of the C3 convertase.

A

Constitutively produced C3b binds to Bb (produced by the action of factor D on factor B). This C3bBb is the C5 convertase.

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29
Q

Complement: Alternative pathway from the formation of the C3 convertase.

A

C3Bb acts on C3 to make more C3b. The resulting C3Bb3b is the C5 convertase.

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30
Q

Complement: Regulators of the alternative pathway.

A

Factor I inactivates C3 unless the latter is bound, for example, to a bacterial cell wall.

Properdin stabilizes the C3 and C5 complexes.

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31
Q

Complement: Activators of the mannan-binding-lectin pathway.

A

Carbohydrates of non-mammalian cells, specifically those of Salmonella, Listeria, Neisseria, Candida, Cryptococcus.

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32
Q

Complement: Mannan-binding-lectin (MBL) pathway to the association with MASPs.

A

Binding of mannose-binding lectin binds to the microbial surface allows MBL to associate with MASPs (MBL-associated serine proteases).

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33
Q

Complement: Mannose-binding-lectin pathway from the association with MASPs.

A

MASP-2 cleaves C4 and C2, thus bypassing C1 and obviating the need for antigen-antibody complexes. C4b2a is the C3 convertase.

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34
Q

Complement: The anaphylotoxins.

A

C3a and C5a, which promote the release of histamine from basophils.

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35
Q

Complement: Rôle of decay-accelerating factor.

A

Accelerates the decay of the C3 and C5 convertases.

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36
Q

Complement: Activators of the alternative pathway.

A

Bacterial cell walls, venoms, endotoxins, complexed IgA.

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37
Q

HLA class I molecules: Components.

A

Heavy chain: Three α domains.

Light chain: β₂-microglobulin.

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38
Q

HLA class I molecules: Distribution on body cells.

A

Found on most nucleated cells.

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39
Q

HLA class I molecules: Function.

A

Mediation of the function of CD8-positive T cells, which kill cells that express foreign antigen in conjunction with their class I molecules.

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40
Q

HLA class II molecules: Components.

A

An α chain and a β chain, each of which has

Two domains similar to the immunoglobulin light chains.

A transmembrane domain.

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41
Q

HLA class II molecules: Distribution on body cells.

A

Found on B cells, antigen-presenting cells, and activated T cells.

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42
Q

Primary immunodeficiencies: Who is mainly affected?

A

Males.

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43
Q

Defects of immunoglobulins or of B cells: Clinical manifestations.

A

Recurrent bacterial infections of the respiratory tract, ultimately leading to bronchiectasis.

Recalcitrant intestinal infections such as with Giardia intestinalis.

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44
Q

Defects in T cells: Clinical manifestations.

A

Viral and fungal infections, e.g. mucocutaneous candidiasis.

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45
Q

Defects of phagocyte: Clinical manifestations.

A

Infections by staphylococci and other catalase-positive organisms.

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46
Q

Defects in terminal components of complement: Clinical manifestations.

A

Severe infections by encapsulated organisms such as N. meningitidis and S. pneumoniae.

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47
Q

Primary immunodeficiencies: Possible findings on physical examination.

A

Large lymph nodes: Common-variable immunodeficiency, chronic granulomatous disease.

Small lymph nodes: Other B-cell deficiencies.

Petechiae, easy bruising: Wiskott-Aldrich syndrome.

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48
Q

How injection of antigens can be used to determine the type of immunological defect.

A

Impaired response to protein antigens (tetanus toxoid, diphtheria toxoid): Defect in T cells, B cells, or both.

Impaired response to carbohydrate antigens (pneumococcal or meningococcal vaccine): Defect in B cells.

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49
Q

RAST:

A. Meaning.
B. Principle.

A

A. Radioallergosorbent test.

B. The amount of IgE production elicited by an antigen is measured.

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50
Q

Specific tests of T-cell function.

A

Flow cytometry.

Tests of delayed-type hypersensitivity.

Proliferation assays.

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51
Q

Proliferation assays for T cells:

A. Agents.
B. What gets measured?

A

A. Concanavalin A, phytohemagglutinin.

B. The uptake of tritiated thymidine.

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52
Q

Tests of NK-cell function.

A

Chromium-release assays.

Colorimetric detection of granzyme B.

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53
Q

Nitroblue tetrazolium test: Principle.

A

A neutrophil capable of generating a normal oxidative burst will reduce yellow NBT to purple-blue formazan.

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54
Q

Nitroblue tetrazolium test: Interpretation.

A

~100% of neutrophils are F+: Normal.

<10%: Chronic granulomatous disease.

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55
Q

Use of flow cytometry to test for oxidative burst in neutrophils.

A

Oxidation of dihydrorhodamine 123 produces fluorescence that can be measured.

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56
Q

CH50 test:

A. Purpose.
B. Method.
C. Reported value.

A

A. To assess function of the classic pathway.

B. One determines the dilution of the patient’s serum that lyses 50% of immunoglobulin-coated sheep erythrocytes.

C. The reciprocal of the dilution.

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57
Q

Causes of deceased C3.

A

Primary deficiency of C3.

Activation of the alternative pathway.

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58
Q

Complement components measured in assessing the function of the classic pathway.

A

C4, C1q.

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59
Q

Uses of the complement-dependent-cytotoxicity assay (3).

A

Detecting HLA antigens or antibodies to them.

Performing HLA crossmatches.

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60
Q

Mixed lymphocyte culture: Purpose.

A

To detect differences in HLA class II antigens between a potential donor and a recipient.

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61
Q

Cross-reactive antigen group: Definition.

A

Group of HLA alleles for which there is serologic cross-reactivity.

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62
Q

Public antigen:

A. Definition.
B. Example.

A

A. A sequence of amino acids shared among many different HLA antigens.

B. HLA-Bw4, HLA-Bw6.

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63
Q

Public antigens: Significance.

A

In someone lacking a public antigen, sensitization can appear as many different HLA antibodies. This can complicate platelet transfusions and organ transplants.

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64
Q

Advantage of PCR in HLA typing.

A

Distinguishes among HLA types more precisely than does serology.

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65
Q

Panel-reactive-antibody tests:

A. Purpose.
B. Principle.

A

A. To help find a donor for a patient needing a transplant.

B. One determines the proportion of the donor population against which the patient’s HLA antibodies will react.

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66
Q

Panel-reactive-antibody tests: Reporting of results.

A

A PRA of 80% means that the patient has about a 20% chance of finding a compatible organ from an unrelated donor.

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67
Q

HLA matching for transplantation: Most important loci.

A

HLA-A, HLA-B, HLA-DR.

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68
Q

How to prevent hyperacute rejection.

A

Use the complement-dependent-cytotoxicity (CDC) assay to detect preformed antibodies against HLA antigens in the potential donor.

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69
Q

Advantages of flow cytometry in the crossmatching of transplants.

A

More sensitive than the CDC assay.

Detects lower titers of complement-dependent and complement-independent antibodies to the donor graft.

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70
Q

Correlation of Luminex technology with other techniques for crossmatching of transplants.

A

MFI (mean fluorescence intensity) . . .

> 5000: Correlates with incompatibility on flow cytometry of T and B cells.

> 10,000: Correlates with a positive CDC assay and the likelihood of hyperacute rejection.

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71
Q

Required compatibilities for transplantation of

A. Kidney.
B. Liver.
C. Heart.
D. Lung.

A

A. ABO, HLA, crossmatch.

B,C,D. Only ABO compatibility is required.

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72
Q

Required HLA compatibility for transplantation of allogeneic progenitor cells.

A

HLA-A, -B, -C, -DRB1.

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73
Q

Hyperacute rejection:

A. Onset.
B. Location of inciting antigens.

A

A. Within hours.

B. On the endothelium of the graft.

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74
Q

Hyperacute rejection: Histology.

A

Fibrin thrombi, platelet thrombi, necrosis, all due to complement-mediated vascular injury

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75
Q

Acute cellular rejection:

A. Onset.
B. Mechanism.

A

A. Evolves over days to weeks.

B. The recipient’s T cells recognize the donor’s HLA antigens as foreign and mount a powerful cytotoxic response.

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76
Q

Acute cellular rejection: Histology.

A

Lymphocytic infiltration of epithelium (tubulitis) and endothelium, along with interstitial edema.

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77
Q

Acute humoral rejection:

A. Onset.
B. Mechanism.

A

A. Within days or weeks.

B. Antibodies against the endothelium of the graft.

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78
Q

Acute humoral rejection: Histology (2).

A

Fibrinoid necrosis and neutrophilic infiltration of vessel walls, resulting in infarction.

  • or -

Subendothelial intimal thickening with more protracted ischemia of the graft.

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79
Q

Acute humoral rejection: Patients at high risk.

A

Those who have been previously immunized against HLA antigens, due to pregnancy or transfusion.

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80
Q

Acute humoral rejection: Immunohistochemistry.

A

Demonstrates deposition of C4d in vessel walls.

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81
Q

Chronic rejection:

A. Onset.
B. Mechanism.

A

A. Develops over months or years.

B. Mediated by both lymphocytes and antibodies.

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82
Q

Chronic rejection: Histology.

A

Interstitial fibrosis, arteriolosclerosis, deposits of complement in peritubular vessels.

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83
Q

Graft-versus-host disease: Required conditions.

A

Immunocompetent T cells from the donor.

Immunosuppressed recipient.

Antigenic differences between donor and recipient.

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84
Q

Acute GVHD:

A. Onset.
B. Affected organs.

A

A. Within 100 days after transplant; usually within 30 days.

B. Skin, intestinal tract, hepatobiliary tract.

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85
Q

Acute GVHD: Effect on skin.

A

Red, itchy rash.

Histology: Apoptosis similar to that found in erythema multiforme.

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86
Q

Acute GVHD: Effect on intestinal tract.

A

Diarrhea.

Histology: Ectatic crypts with attenuated enterocytes, crypt abscesses, and apoptosis.

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87
Q

Acute GVHD: Effect on hepatobiliary tract.

A

Jaundice.

Histology: Mononuclear infiltrate in portal areas, with endothelialitis, ductitis, ductopenia.

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88
Q

Chronic GVHD:

A. Onset.
B. Affected organs.

A

A. >100 days after transplant.

B. Skin, intestinal tract, hepatobiliary tract; mucosa of mouth, vagina, eye, respiratory tract.

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89
Q

Chronic GVHD: Effect on

A. Skin.
B. Esophagus.
C. Eyes.

A

A. Extensive sclerosis.

B. Strictures.

C. Scarring lesions.

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90
Q

Bruton’s agammaglobulinemia:

A. Onset.
B. Infections.
C. Other complications.

A

A. Around 6 months of age.

B. Bacterial, enteroviral; viral hepatitis.

C. Lymphoid neoplasms, autoimmune diseases.

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91
Q

Bruton’s agammaglobulinemia: Laboratory findings.

A

Marked reduction in serum immunoglobulins.

Lack of CD19-positive B cells.

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92
Q

Bruton’s agammaglobulinemia: Physical examination.

A

Rudimentary lymph nodes and tonsils.

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93
Q

Bruton’s agammaglobulinemia: Gene and its location.

A

BTK on the X chromosome.

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94
Q

Common-variable immunodeficiency:

A. Onset.
B. Infections.
C. Other complications.

A

A. Second or third decade.

B. Recurrent infections of upper and lower respiratory tract; giardiasis.

C. Bronchiectasis, intestinal bacterial overgrowth.

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95
Q

Common-variable immunodeficiency: Laboratory findings.

A

Decreased serum immunoglobulins.

Variable T-cell deficiency.

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96
Q

Common-variable immunodeficiency: Histology.

A

Lack of plasma cells.

Reactive follicular hyperplasia; hyperplastic germinal centers.

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97
Q

Common-variable immunodeficiency: Underlying defect.

A

Excess of Th1 cells -> overproduction of IL-12 -> suppression of Th2 cells -> decreased stimulation of B cells to produce antibody.

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98
Q

Selective IgA deficiency:

A. Incidence.
B. Infections.
C. Other complications.

A

A. 1 in 700.

B. Respiratory and gastrointestinal; many patients have none.

C. Autoimmune disease; anaphylactic transfusion reaction.

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99
Q

Hyper-IgE syndrome: Clinical features.

A

Recurrent staphylococcal infections, coarse facial features, eczema, eosinophilia.

100
Q

Hyper-IgE syndrome: Inheritance.

A

Autosomal dominant or autosomal recessive.

101
Q

Immunodeficiencies with abnormal levels of IgE.

A

Increased: Hyper-IgE, Wiskott-Aldrich, Nezelof’s.

Decreased: Bruton’s, ataxia-telangiectasia.

102
Q

DiGeorge’s syndrome: Embryological defect.

A

Failure of development of the 3rd and 4th pharyngeal pouches.

103
Q

DiGeorge’s syndrome: Physical features.

A

Bifid uvula.

Anomalies of great vessels.

Typical facies.

Hypoplastic thymus and parathyroid.

Esophageal atresia.

104
Q

CHARGE sequence.

A

A form of partial DiGeorge’s syndrome with

Coloboma.
Heart defect.
Atresia, choanal.
Retarded development.
Genital hypoplasia.
Ear abnormalities.
105
Q

DiGeorge’s syndrome:

A. Infections.
B. Other complications.

A

A. Opportunistic fungi, viruses, P. jiroveci.

B. Increased risk of transfusion-related GVHD.

106
Q

DiGeorge’s syndrome: Histology.

A

Lymph nodes: Depleted paracortical regions.

Spleen: Poorly developed periarteriolar lymphatic sheaths.

107
Q

DiGeorge’s syndrome:

A. Genetic basis.
B. Etiology.

A

A. Microdeletions involving 22q11.2.

B. Usually sporadic; some cases associated with isotretinoin.

108
Q

Severe combined immunodeficiency: Affected parts of the immune system.

A

Function of T cells.

Immunoglobulin levels.

Thymus (dysplastic).

109
Q

Severe combined immunodeficiency:

A. Clinical manifestations.
B. Genetic basis.

A

Life-threatening immunodeficiency.

Increased risk of transfusion-related GVHD.

110
Q

Hyper-IgM syndrome: Clinical and laboratory findings.

A

Clinically similar to Bruton’s agammaglobulinemia.

Increased IgM; decrease in other immunoglobulins.

111
Q

Hyper-IgM syndrome:

A. Molecular defect.
B. Inheritance.

A

A. Defect in CD154, the T-cell ligand for the B-cell receptor CD40.

B. X-linked.

112
Q

Wiskott-Aldrich syndrome: Clinical triad.

A

Eczema, thrombocytopenia, immunodeficiency.

113
Q

Wiskott-Aldrich syndrome:

A. Pathogens.
B. Gene.

A

A. Pneumococci and other encapsulated bacteria, Pneumocystis jiroveci, herpesviruses.

B. WAS gene on the X chromosome.

114
Q

Ataxia-telangiectasia:

A. Sites of infection.
B. Other complication.

A

A. Upper and lower respiratory tract.

B. Various malignancies.

115
Q

Ataxia-telangiectasia:

A. Affected parts of the immune system.
B. Laboratory abnormalities.

A

A. T cells and B cells.

B. Deficient IgA; very high serum AFP and CEA.

116
Q

Ataxia-telangiectasia:

A. Inheritance.
B. Gene.

A

A. Autosomal recessive.

B. ATM gene on 11q23.3.

117
Q

Chronic mucocutaneous candidiasis:

A. Affected part of the immune system.
B. Clinical associations.

A

A. T-cell function.

B. Endocrine dysfunction, autoimmune disorders.

118
Q

Chronic mucocutaneous candidiasis: Gene.

A

Autoimmune-regulator gene (AIRE) on 21q22.3.

119
Q

Duncan’s disease:

A. Type of immunodeficiency.
B. Pathogens.
C. Laboratory abnormalities.

A

A. Often similar to that of common-variable immunodeficiency.

B. Epstein-Barr virus can cause fulminant or fatal infection.

C. Inverted ratio of CD4 to CD8, hypogammaglobulinemia.

120
Q

Duncan’s disease: Gene.

A

SH2D1A gene on Xq25 (encodes part of SLAM-associated protein).

121
Q

Chronic granulomatous disease:

A. Most common enzymatic defect.
B. Pathogens.
C. Abnormalities of cell-surface antigens.

A

A. NADPH oxidase.

B. Staphylococci, enterobacters, Aspergillus.

C. Leukocytes lack C3bR; red cells often lack Kx.

122
Q

Chédiak-Higashi syndrome:

A. Inheritance.
B. Gene.

A

A. Autosomal recessive.

B. CHS1/LYST (lysosomal trafficking regulator) on 1q42.1-2.

123
Q

May-Hegglin anomaly:

A. Inheritance.
B. Peripheral smear.

A

A. Autosomal dominant.

B. Döhle-body-like inclusions that consist of RNA; variably sized platelets; thrombocytopenia.

124
Q

Alder-Reilly anomaly:

A. Peripheral smear.
B. Clinical association.

A

A. Large azurophilic granules in all leukocytes.

B. Mucopolysaccharidoses.

125
Q

Pelger-Huët anomaly:

A. Functional abnormality.
B. Inheritance.
C. Finding in homozygotes.

A

A. None.

B. Autosomal dominant.

C. Neutrophils with one nuclear lobe (Stodtmeister cells).

126
Q

Jordan’s anomaly.

A

Fat vacuoles in neutrophils.

127
Q

Deficiency in complement components that is associated with lupus.

A

C1q, C2, C4.

128
Q

Deficiency in complement components that is associated with recurrent infections with Gram-positive organisms.

A

C2, C3.

129
Q

Deficiency in complement components that is associated with serious systemic infections, esp. by neisseriae.

A

C5-C9.

130
Q

Hereditary angioedema:

A. Inheritance.
B. Molecular basis.

A

A. Autosomal dominant.

B. Deficiency of the inhibitor of C1 esterase.

131
Q

Hereditary angioedema: Laboratory findings (2).

A

During attacks: High urinary histamine, high serum C1; low serum CH50, C4, and C2.

Between attacks: Low serum C4, normal C2.

132
Q

Hereditary angioedema: Therapy (3).

A

First-line: Recombinant C1-esterase inhibitor.

Second-line: Ecallantide (inhibits kallikrein), icatibant (blocks receptor of bradykinin B2).

Prophylaxis: Androgenic agents.

133
Q

HLA type associated with

A. Hereditary hemochromatosis.
B. 21-hydroxylase deficiency.
C. Behçet’s disease.

A

A. A3.
B. C4.
C. B51.

134
Q

HLA type associated with

A. Celiac disease.
B. Multiple sclerosis.
C. Diabetes mellitus, type 1.

A

A. DQ2, DQ8.
B. DRB1, DQB1.
C. DR3, DR4.

135
Q

HLA type associated with

A. Narcolepsy.
B. Rheumatoid arthritis.

A

A. DQ6.

B. DR4.

136
Q

Autoimmune disease that can result from infection by HBV.

A

Polyarteritis nodosa.

137
Q

Associations between drugs and autoimmune diseases (5).

A

Methyldopa: Autoimmune hemolytic anemia.

Penicillamine: Systemic vasculitis.

Procainamide, hydralazine, isoniazid: Drug-induced lupus.

138
Q

LE cell:

A. Definition.
B. Appearance.

A

A. A neutrophil that has ingested the nucleus of a lymphocyte.

B. The lymphocyte nucleus should appear pink and without discernible chromatin.

139
Q

LE cell:

A. Locations (3).
B. Variant.

A

A. Pleural effusions, joint effusions, bone-marrow aspirates.

B. Tart cell: The lymphocyte nucleus appears blue-purple, with retained chromatin detail.

140
Q

Direct immunofluorescence: Purpose and technique.

A

To look for autoantibodies that are already bound to the tissues.

Cryostat sections of patient’s tissue are incubated with fluorescein-labeled antihuman globulin.

141
Q

Indirect immunofluorescence: Purpose and technique.

A

To look for circulating autoantibodies.

The patient’s serum is incubated with cells or tissues containing known antigens. Then fluorescein-labeled antihuman globulin is added.

142
Q

ANA: How detected.

A

By means of indirect immunofluorescence with HEp-2 cells.

143
Q

ANA: How measured.

A

The pattern of fluorescence is noted for mitotically active and inactive cells.

All positive samples are serially diluted to determine titer.

144
Q

ANA with homogeneous staining: Antigens.

A

ssDNA, histones.

145
Q

ANA with homogeneous staining: Diseases.

A

High titer: SLE.

Low titer: Sjögren’s syndrome, MCTD, RA.

146
Q

ANA with peripheral (rim) staining: Antigens.

A

dsDNA (nuclear envelope).

147
Q

ANA with peripheral (rim) staining: Diseases.

A

Primary biliary cirrhosis, chronic liver disease, drug-induced lupus.

148
Q

ANA with homogeneous or peripheral (rim) staining: How to distinguish from a speckled pattern.

A

In the speckled pattern of ANA, the mitotic figures are not stained.

149
Q

ANA with speckled pattern: Antigens.

A

Extractable nuclear antigens: Sm, RNP, Scl-70, Ro/SS-A, La/SS-B, PCNA.

150
Q

ANA with speckled pattern: Diseases.

A

CREST syndrome, scleroderma, SLE, MCTD, Sjögren’s syndrome, rheumatoid arthritis.

151
Q

ANA with nucleolar pattern: Antigens.

A

RNA polymerase I, small RNPs, Th/To, B23, PM-Scl, NOR-90.

152
Q

ANA with nucleolar pattern: Diseases.

A

Scleroderma, polymyositis/scleroderma.

153
Q

ANA with centriolar pattern: Antigens.

A

CENP-A, -B, and -C.

154
Q

ANA with centriolar pattern: Diseases.

A

CREST syndrome, idiopathic Raynaud’s phenomenon.

155
Q

ANA: Specificity for lupus (2).

A

80% at a titer of 1 : 40.

95% at a titer of 1 : 160.

156
Q

How indirect immunofluorescence is used to test for antibodies to double-stranded DNA.

A

The kinetoplast of Crithidia luciliae contains double-stranded DNA.

157
Q

Systemic lupus erythematosus: Most important diagnostic tests (4).

A

ANA.

Antibodies against Sm, dsDNA, phospholipids.

158
Q

Antibodies to RNP: Disease.

A

MCTD if of high titer (1 : 10,000) and in isolation.

159
Q

Ro and La antibodies:

A. Synonyms.
B. Disease.

A

Sjögren’s syndrome, if there are no antibodies against dsDNA or Sm.

160
Q

Antibodies to Scl-70: Disease.

A

Progressive systemic sclerosis.

161
Q

Antibodies to centromeres: Diseases.

A

CREST syndrome.

Occasionally seen in progressive systemic sclerosis and Raynaud’s phenomenon.

162
Q

Signal-recognition particles:

A. Examples.
B. Antibodies are associated with what disease?

A

A. Jo-1, PM/Scl.

B. Inflammatory myositis.

163
Q

Screening for antibodies to cytoplasmic antigens: Substrate.

A

Rat tissue: Liver, kidney, and stomach.

164
Q

Antimitochondrial antibodies:

A. Reactivity.
B. Specificity.

A

A. Gastric parietal cells, renal tubular cells, hepatocytes.

B. M2 antigen.

165
Q

Anti-smooth-muscle antibody: Reactivity.

A

Smooth muscle of gastric tissue and renal arterioles.

166
Q

Anti-liver-kidney-microsomal antibody: Reactivity.

A

Hepatocytes, renal tubular cells.

167
Q

Antineutrophil-cytoplasmic antibodies: Procedure (2).

A

The patient’s serum is incubated with alcohol-fixed neutrophils.

All positive samples are tested for ANA in order to exclude a false-positive ANCA.

168
Q

Cytoplasmic ANCA:

A. Pattern.
B. Specificity.
C. Disease.

A

A. Cytoplasmic granular pattern with perinuclear accentuation.

B. Proteinase 3.

C. Wegener’s granulomatosis.

169
Q

Perinuclear ANCA:

A. Pattern.
B. Specificity.
C. Diseases (4).

A

A. Mostly perinuclear.

B. Myeloperoxidase.

C. Microscopic polyangiitis, polyarteritis nodosa, PSC, ulcerative colitis.

170
Q

Technique other than immunofluorescence that is being used to identify autoantibodies in tissues.

A

Enzymatic immunoassay (EIA).

171
Q

Anti-Jo1:

A. Synonym.
B. Disease.

A

A. Anti-tRNA synthetase.

B. Polymyositis, especially with pulmonary fibrosis.

172
Q

Antihistone antibodies: Disease.

A

Drug-induced lupus.

173
Q

Antibodies to extractable nuclear antigens: Substrate for testing.

A

Calf thymus.

174
Q

Anti-RNP antibodies: Disease.

A

Mixed connective-tissue disease.

175
Q

Antinucleolar antibodies: Disease.

A

Scleroderma.

176
Q

Antibodies to SS-A and SS-B: Diseases (3).

A

Sjögren’s syndrome,

Neonatal lupus.

Lupus nephritis – if ANA and anti-SS-A are positive but anti-SS-B is negative.

177
Q

Antimicrosomal antibodies: Disease.

A

Highly specific and sensitive for Hashimoto’s thyroiditis.

Present in up to half of patient’s with Grave’s disease.

178
Q

Antibodies to the glomerular basement membrane:

A. Specificity.
B. Disease.

A

A. Type IV collagen.

B. Goodpasture’s syndrome.

179
Q

Antibodies to intermediate filaments: Disease.

A

Polymyositis/dermatomyositis.

180
Q

Antibody to RNA polymerase: Disease.

A

Scleroderma.

181
Q

Antiribosomal P antibodies: Disease.

A

Polymyositis/dermatomyositis.

182
Q

Antibody to topoisomerase:

A. Disease.
B. Synonym.

A

A. Scleroderma.

B. Anti-Scl-70.

183
Q

Anti-U1-RNP:

A. Disease.
B. Synonym.

A

A. Progressive systemic sclerosis.

B. Anti-fibrillarin.

184
Q

Utility of test for antibodies to cyclic citrullinated peptides.

A

More specific and more sensitivity than rheumatoid factor.

185
Q

Causes of elevated angiotensin-converting enzyme (4).

A

Sarcoidosis.

Primary biliary cirrhosis.

Gaucher’s disease.

Leprosy.

186
Q

SLE: Findings that occur in more than half of patients.

A

Rash.

Arthralgia.

Neurological disease.

Serositis.

Hematological disease.

187
Q

SLE: Criteria of the American College of Rheumatology.

A 
Malar rash, discoid rash, photosensitivity.
Oral ulcers.
Arthritis, serositis.
Renal dysfunction.
Neurological dysfunction.
Hematological disorder.
Autoantibodies.
Antinuclear antibody.
188
Q

SLE: Most common autoantibodies (6).

A

ANA.

Anti-dsDNA.

Anti-ssDNA.

Anti-SS-A, anti-SS-B.

Anti-Sm.

189
Q

Drug-induced lupus: Drugs.

A

Procainamide.

Hydralazine.

Quinidine.

Isoniazid.

IFN-α.

190
Q

Drug-induced lupus: Specificity of antibody.

A

H2A-H2B complex.

191
Q

Rheumatoid arthritis: Hematological findings.

A

Anemia of chronic disease.

Thrombocytosis.

192
Q

Rheumatoid arthritis: How many have a rheumatoid factor?

A

Most, but up to 30% have none initially.

193
Q

Rheumatoid arthritis: Synovial fluid.

A

Pleocytosis, mostly neutrophils.

Decreased glucose.

194
Q

The seronegative spondyloarthropathies.

A

Ankylosing spondylitis.

Psoriatic arthritis.

Reactive arthritis.

195
Q

Reactive arthritis: Pathogens.

A

Chlamydia.

Shigella, Salmonella, Yersinia, Campylobacter.

196
Q

Celiac disease: Associated diseases of autoimmunity.

A

Diabetes mellitus, type 1.

Autoimmune liver disease.

197
Q

Celiac disease: Associated non-immunological diseases.

A

IgA deficiency.

Cystic fibrosis.

Turner’s syndrome.

Down’s syndrome.

198
Q

Celiac disease: Possible nutrient deficiencies.

A

Iron, folate.

199
Q

Celiac disease: Extraintestinal manifestations.

A

Dermatitis herpetiformis.

Arthritis.

200
Q

Progressive systemic sclerosis:

A. Synonym.
B. Leading causes of death.

A

A. Scleroderma.

B. Pulmonary hypertension, renal crisis.

201
Q

IgG4-related systemic sclerosis: Manifestations (6).

A 
Sclerosing mediastinitis.
Sclerosing pancreatitis.
Sclerosing cholangitis.
Riedel's thyroiditis.
Idiopathic retroperitoneal fibrosis. 
Orbital pseudotumor.
202
Q

IgG4-related systemic sclerosis:

A. Histology.
B. Other test.

A

A. Infiltrate of IgG4-producing plasma cells.

B. Serum IgG4.

203
Q

Autoimmune hepatitis: Types and their antibodies.

A

Type 1: ANA, anti-smooth-muscle, anti-soluble liver protein.

Type 2: Anti-liver-kidney-microsomal, anti-CYP 2D6.

204
Q

Progressive systemic sclerosis: Types (3).

A

Diffuse-cutaneous: Involves skin and viscera.

Limited-cutaneous: Like D-C but less severe.

Localized: Limited to skin.

205
Q

Primary biliary cirrhosis: Antibodies.

A

Antimitochrondrial.

IgG anti-sp100.

IgG anti-gp210.

206
Q

Primary biliary cirrhosis: Non-immunological laboratory abnormality.

A

Hypercholesterolemia.

207
Q

Elevated immunoglobulin in

A. Autoimmune hepatitis.
B. Primary biliary cirrhosis.

A

A. IgG.

B. IgM.

208
Q

Rheumatoid arthritis: Extraarticular manifestations (4).

A

Interstitial lung disease.

Pleuritis.

Endocarditis.

Systemic vasculitis.

209
Q

Sjögren’s syndrome: Associations (4).

A

Rheumatoid arthritis.

Primary biliary cirrhosis.

SLE.

Systemic sclerosis.

210
Q

Sjögren’s syndrome: Histologic diagnosis.

A

Biopsy of minor salivary gland showing >1 inflammatory focus per 4 mm².

A “focus” is an aggregate of at least 50 mononuclear inflammatory cells.

211
Q

Vasculitides having no serologic markers (6).

A 
Giant-cell arteritis.
Takayasu's arteritis.
Kawasaki syndrome.
Cogan's syndrome.
Behçet's disease.
Buerger's disease.
212
Q

Vasculitides with pANCA (2).

A

Microscopic polyangiitis.

Churg-Strauss syndrome.

213
Q

Vasculitis with cANCA.

A

Wegener’s granulomatosis.

214
Q

Immunoglobulin light chains: Domains.

A

One variable domain and one constant domain.

215
Q

Kawasaki’s disease:

A. Age at onset.
B. Mortality.

A

A. 2 or 3 years.

B. 1-2% of untreated cases; death results from coronary-artery disease.

217
Q

Serologic marker: Polyarteritis nodosa.

A

Some patients are HBV positive; otherwise no markers.

218
Q

Kawasaki’s disease:

A. When does the desquamation occur?
B. Other clinical features (4).

A

A. After 5 days of fever.

B. Bilateral conjunctivitis, cracking of lips, strawberry tongue, cervical lymphadenopathy.

219
Q

Henoch-Schönlein purpura: Renal biopsy.

A

Findings similar to those of IgA nephropathy.

220
Q

Cogan’s syndrome: Clinical manifestations.

A

Keratitis.

Vestibulo-auditory symptoms.

221
Q

Henoch-Schönlein purpura: Clinical features (3).

A

Palpable purpura on lower extremities.

Abdominal pain.

Glomerulonephritis.

223
Q

Polyarteritis nodosa: Affected organs.

A

Many, but the lungs are spared.

224
Q

Polyarteritis nodosa: Neurological manifestation.

A

Mononeuritis complex.

225
Q

Polyarteritis nodosa: Histology.

A

Segmental fibrinoid necrosis of inflamed arterial walls, especially at branch points.

Active and healing lesions are seen in the same biopsy.

226
Q

Microscopic polyangiitis:

A. Involved organs.
B. Involved vessels.

A

A. Many, but especially lungs and kidneys.

B. Small arterioles and venules.

227
Q

Microscopic polyangiitis:

A. Typical renal lesion.
B. Serologic marker.

A

A. Focal-segmental necrotizing glomerulonephritis, pauci-immune type.

B. pANCA.

228
Q

Chung-Strauss syndrome: Clinical presentation.

A

Asthma of adult onset; progression to fulminant vasculitis.

229
Q

Churg-Strauss syndrome: Main cause of death.

A

Cardiac disease.

230
Q

Churg-Strauss syndrome: Laboratory findings.

A

Peripheral eosinophilia.

pANCA.

231
Q

Behçet’s disease: Classic triad.

A

Oral aphthous ulcers.

Genital aphthous ulcers.

Uveitis.

232
Q

Behçet’s disease: Unique test.

A

Pathergy test: One looks for development of erythema, induration, and pustule at the site of a needle stick.

233
Q

Giant-cell arteritis: Association.

A

Polymyalgia rheumatica.

234
Q

Dermatomyositis/polymyositis: Typical serologic markers (3).

A

Anti-Jo1, anti-SRP, anti-PM/Scl.

235
Q

Inclusion-body myositis:

A. Incidence.
B. Diagnosis.

A

A. Most common inflammatory myopathy in older adults.

B. Muscle biopsy. No specific serologic markers.

236
Q

Myasthenia gravis: Antibody and its sensitivity and specificity.

A

Antibody to the acetylcholine receptor.

75-95% sensitive.

~100% specific.

237
Q

Myasthenia gravis: Types in which anti-titin antibodies are found.

A

Late-onset.

Thymoma-associated.

238
Q

Myasthenia gravis: Types in which anti-MuSK antibodies are found.

A

Seronegative.

239
Q

Familial Mediterranean fever:

A. Inheritance.
B. Gene and its location.
C. Affected protein.

A

A. Autosomal recessive.

B. MEFV on chromosome 16.

C. Pyrin (marenostrin).

240
Q

Familial Mediterranean fever:

A. Symptoms.
B. Complication.

A

A. Fever; pain from serositis.

B. Renal amyloidosis (AA).

241
Q

Type I hypersensitivity:

A. Synonym.
B. Mechanism.

A

A. Immediate.

B. IgE-mediated release of histamine, serotonin, and other substances from mast cells.

242
Q

Diagnosis of anaphylaxis:

A. Use of tryptase.

B. Use of histamine.

A

A. Mature serum tryptase better reflects mast-cell degranulation than does total tryptase.

B. Plasma histamine peaks earlier than tryptase and returns to normal in 1 hour. Urine tryptase may remain elevated for 24 hours.

243
Q

Type II hypersensitivity:

A. Synonym.
B. Mechanism.

A

A. Antibody-mediated cellular cytotoxicity.

B. Formation of antibody-antigen complexes, opsonization.

244
Q

Type II hypersensitivity: Examples.

A

Goodpasture’s syndrome, myasthenia gravis, immune-mediated hemolysis.

245
Q

Takayasu’s arteritis:

A. Histology.
B. Distinction from giant-cell arteritis.

A

A. Infiltration of arterial wall by mononuclear inflammatory cells and multinucleate giant cells.

B. Takayasu’s arteritis affects patients under 40.

246
Q

Type IV hypersensitivity: Mechanism.

A

Response of T cells to antigen, usually with granulomatous inflammation.

247
Q

Type III hypersensitivity:

A. Mechanism.
B. Examples.

A

A. Formation of antibody-antigen complexes, activation of complement.

B. Serum sickness, Henoch-Schönlein purpura.

251
Q

Wegener’s granulomatosis: Typical renal lesion.

A

Focal-segmental necrotizing glomerulonephritis, pauci-immune type.

261
Q

Takayasu’s arteritis: Possible clinical mimic.

A

Syphilitic arteritis.

CP Reviewer 2 (8 decks)

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