Msi Week 3: Lab Testing Part2

Front 1

Autoantibodies are generally what class of immunoglobulins?

Do B-cell need help from T cells to produce/secrete this class of immunoglobulins?

Back 1

IgG class

Yes, T-cell help (in the germinal center) is required for Ig class switching

Front 2

Group of autoantibodies that react with a variety of nuclear antigens.

Back 2

Antinuclear antibodies (ANA)

Front 3

What type of autoantibodies are hallmarks of systemic autoimmune diseases?

Back 3

Antinuclear antibodies (ANAs)

Front 4

Are autoantibodies diagnostic for specific diseases?

Back 4

No. Apart from rheumatic disorders, they may be found in normal population and with other conditions.

*Therefore, only test when clinically indicated

Front 5

What specific type of test is run to check for ANAs?

Back 5

Indirect immunofluorescence ANA test (IF)

Front 6

Which test modifies the immunodiffusion technique to generate somewhat greater sensitivity?

Back 6

Counterimmunoelectrophoresis (CIE)

*Immunodiffusion has low sensitivity and requires a long time, but has increased specificity

Front 7

Which autoantibody test is highly sensitive and rapid, and has low specificity?

Is this test easy to apply to a clinical setting?

Back 7

Enzyme Linked Immunoabsorbent Assays (ELISA)

This test can be very confusing when adapted to clinical practice as the significance of low-titer antibodies in the general population are unknown.

Front 8

What is the most widely used method for ANA detection?

Is this test highly sensitive or highly specific?

Back 8

Indirect immunofluorescence ANA test (IF)

Highly SENSITIVE and rapid.

Front 9

What ANA titer is considered clinically significant?

Back 9

>1:120

Front 10

List 5 patterns that can be seen in an IF test.

Back 10

1. Speckled
2. Homogenous
3. Centromere
4. Nucleolar
5. Rim/peripheral

Front 11

Are Hep 2 cells in IF derived from human or animal cell lines?

Back 11

Human cell lines
(ensures better specificity than animal tissues)

Front 12

If an ANA test came back negative for a patient, is it fairly safe assume that the patient does not have SLE?

Back 12

ANA tests are highly sensitive for SLE, so if negative, the chances of the patient having SLE are highly unlikely

Front 13

Do strong positive ANA titers have a larger or smaller dilution (higher or smaller denominator)?

Back 13

Larger dilution (higher denominator)

(Ex: 1:1280 is a strong positive)

Front 14

ANA titers in rheumatic disease patients often times exceed what ratio?

Back 14

1:1000

Front 15

Do titers correlate with disease activity?

Back 15

No.

Front 16

Which ANA pattern occurs due to antibodies directed against DNA and is specific for SLE?

Back 16

Rim/peripheral

Front 17

Which ANA pattern is specific for Limited systemic sclerosis and occurs due to antibodies directed against centromeres?

Back 17

Centromere

Front 18

Which ANA pattern is relatively specific for scleroderma (Diffuse systemic sclerosis)?

Back 18

Nucleolar

Front 19

Which ANA pattern is nonspecific and occurs due to antibodies directed against histones?

Back 19

Diffuse/homogenous

Front 20

Which ANA pattern is nonspecific and occurs due to antibodies directed against "extractable nuclear antigens"?

Back 20

Speckled

*Most common pattern

Front 21

Which autoantibodies appear in approximately 73% of SLE patients?

What clinical condition shows a strong correlation to this antibody?

Back 21

anti-dsDNA

Strongly correlate with nephritis

Front 22

T or F.

Anti-Sm is nearly only found in SLE

Back 22

TRUE.

Front 23

Which two autoantibodies are directed against spliceosomal protiens?

Back 23

1. Anti-Smith (Sm)
2. Anti-Ribonucleoprotein (RNP)

Front 24

If found alone, which autoantibody generally results in a more milder form of SLE (usually absence of renal involvement)?

Back 24

Anti-nRNP

Front 25

List 5 clinical features associated w/ anti-RNPs.

Back 25

1. Myositis
2. Raynaud's phenomenon
3. Athralgia/athritis
4. Esophaeal dysmotility
5. Sclerodactyly

Front 26

Which autoantibodies are highly frequent in patients with Sjogren's syndrome and SLE?

Back 26

Anti-Ro/SSA and Anti-La/SSB

(also high prevalence in RF positivity)

Front 27

T or F.

Anti-La is often seen without anti-Ro antibodies.

Back 27

FALSE.

Anti-La is rarely seen without anti-Ro antibodies

Front 28

Which autoantibody is associated with photosensitive skin rash, subacute cutaneous lupus erythematosus, neonatal lupus syndrome, and C2, C4 deficiencies?

Back 28

Anti-Ro/SSA antibodies

Front 29

Which antibody is associated with pulmonary disease, lymphopenia and thrombocytopenia (within SLE), cardiac fibroelastosis in congenital heart block and primary biliary cirrhosis?

Back 29

Anti-Ro/SSA

Front 30

Which autoantibody is found in high titers in Sjogren's syndrome and have greater incidence of extra glandular features, especially purpura and vasculitis?

Back 30

Anti-Ro/SSA

Front 31

Which autoantibody is present in 87% of Sjogren's syndrome patients and participates in the termination of transcription of RNA polymerase III?

Back 31

Anti-La/SSB

Front 32

Which autoantibody is associated with neurologic involvement and rheumatoid factor positivity in SLE and SS?

Back 32

Anti-La/SSB

Front 33

What type of autoantibodies are present in vasculitic syndromes?

Back 33

Antineutrophil cytoplasmic antibodies (ANCAs)

*Autoantibodies to the cytoplasmic consituents of granulocytes

Front 34

Antibodies to proteinase-3

Back 34

c-ANCA
(antineutrophil cytoplasmic antibody)

Front 35

Antibodies to myeloperoxidase

Back 35

p-ANCA
(antineutrophil cytoplasmic antibody)

Front 36

Which autoantibody is 98% specific for Wegener's granulomatosis, microscopic polyarteritis, or idiopathic crescentic glomerulonephritis?

Back 36

c-ANCA

*Often precede disease activity and may guide treatment

Front 37

List 3 systemic sclerosis autoantibodies.

Back 37

1. Anti-nucleolar
2. Anti-centromere
3. Anti-Scl-70

Front 38

Which autoantibody is common in systemic sclerosis, but can also be found in limited scleroderma?

Back 38

Anti-nucleolar

Front 39

Which autoantibody is found almost exclusively in patients w/ limited cutaneous systemic sclerosis?

Back 39

Anti-centromere

*Associated with an increased risk of malignancy

*Associated with CREST syndrome:
Calcinosis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactily
Telangiectasia

Front 40

What are the clinical features of CREST syndrome and what disease is it associated with?

Back 40

CREST syndrome is associated with Limited cutaneous systemic sclerosis.

1. Calcinosis
2. Raynaud's phenomenon
3. Esophageal dysmotility
4. Sclerodactyly
5. Telangiectasia

Front 41

Which autoantibody is present in 22-40% of slceroderma patients and targets the catalytic carboxyl-terminal region of DNA topoisomerase I?

Back 41

Anti-Topoisomerase-1 (Scl-70)

(Predicts diffuse cutaneous disease and proximal skin involvement, longer disease duration, cancer, pulmonary fibrosis, digital pitting scars, cardiac involvement).

Front 42

Which autoantibodies are sensitive, but non-specific for drug-induced lupus?

Back 42

Anti-histone antibodies

Front 43

Which autoantibody has been variably associated w/ neuropsychiatric lupus, including psychosis?

Back 43

Antiribosomal P (Ribo P) antibodies

Front 44

Which autoantibody is associated with autoimmune hepatitis?

What does a positive titer >1:80 indicate?

Back 44

Smooth muscle antibody (SMA)

Positive titer >1:80 can be associated with Chronic active hepatitis

Titers <1:80 can be associated with:
1. Primary biliary cirrhosis
2. Epstein barr virus
3. Cytomegalovirus

Front 45

What type of antigens are usually attacked by autoantibodies involved in inflammatory muscle diseases?

Back 45

cytoplasmic antigens (90%)

Front 46

Do the majority of DM/PM patients test positively for ANA?

Back 46

Yes. 40-80%

Front 47

List 3 myositis-specific autoantibodies.

Back 47

1. Anti-synthetase antibodies (Anti-Jo-1, Non-Jo-1)
2. Anti-signal recognition particle (SRP)
3. Anti-Mi-2

*All associated with DM/PM

Front 48

Which autoantibody targets class II histidyl tRNA synthetase, correlates with the "antisynthetase syndrome," and is most common in PM/DM populations?

Back 48

Anti-Jo-1

Antisynthetase syndrome:
1. Interstitial lung disease
2. Arthritis
3. Raynaud's phenomenon
4. Mechanics hands
5. Hyperkerototic lines
6. Sclerodactyly
7. Facial telangiectasia
8. Calcinosis
9. Sicca

Front 49

Which autoantibody is associated with polymyositis (5%) and with acute onset, severe disease, resistance to therapy, and cardiac involvement?

Back 49

Anti-SRP (autoantibody associated w/ inflammatory muscle disease)

*Poorest overall prognosis = much worse than Anti-Jo-1, Anti-Mi-2, Anti-PM-Scl, Anti-nRNP

Front 50

Which autoantibody is much more prevalent in DM patients than PM patients?

Back 50

Anti-Mi-2
(95% of patients with Anti-Mi-2 have DM rather than PM)

Front 51

Which autoantibody is associated with inflammatory muscle diseases and generally shows greater dermatologic involvement?

Back 51

Anti-Mi-2

(Shawl (or V) rash is often apparent in DM patients. It is a diffuse, flat, erythematous lesion over the back and shoulders or in a "V" over the posterior neck and back or neck and upper chest, worsens with UV light)

Front 52

Which autoantibody is associated with myositis-scleroderma overlap without SLE features?

Back 52

Anti-PM-Scl

(Associated w/ arthritis, DM skin lesions, mechanic's hands, eczema, and calcinosis)

Front 53

What syndrome is present in 30% of SLE patients and results in arterial and venous thromboses, recurrent miscarriages, and thrombocytopenia?

Back 53

Antiphospholipid syndrome

(Associated with anti-cardiolipins, lupus anticoagulant, and beta 2 glycoprotein 1)

Front 54

List 3 autoantibodies associated with Antiphospholipid syndrome.

Back 54

1. Anti-cardiolipins
2. Lupus anticoagulant
3. Beta 2 glycoprotein 1

Front 55

Which autoantibodies are IgG and IgM isotypes, and are part of the criteria for the diagnsosis of anti-phospholipid syndrome?

Back 55

Anti-cardiolipin

Front 56

Which antiphospholipid antibodies are associated with thromboembolic events and fetal loss?

Back 56

Anti-cardiolipin
Lupus anticoagulant

Front 57

Which autoantibody is specific against phospholipids in the cell surface, and prolongs the time it takes blood to clot?

Back 57

Lupus anticoagulant

Front 58

The binding of anti-cardiolipin to cardiolipin is dependent on the existence of what glycoprotein?

Back 58

Beta 2 Glycoprotein 1 (B2GP1)

*Part of the criteria fro the diagnosis of anti-phospholipid syndrome

Front 59

A positive ANA test demonstrating a peripheral/rim pattern might be indicative of what autoimmune disease?

Back 59

SLE

(Anti-dsDNA)

Front 60

A positive ANA test demonstrating a diffuse/homogenous pattern might be indicative of what autoimmune diseases?

Back 60

1. SLE
2. drug-induced SLE
3. RA

(Anti-nucleoprotein, anti-histone)

Front 61

A positive ANA test demonstrating a centromere pattern might be indicative of what disease?

Back 61

Limited cutaneous systemic scleroderma (CREST)

(Anti-centromere)

Front 62

A positive ANA test demonstrating a nucleolar pattern might be indicative of what disease?

Back 62

Scleroderma (Raynaud's phenomenon)

(Anti-nucleolar)

Front 63

What two diseases are associated with a speckled ANA pattern and Anti-Ro and Anti-La antibodies?

Back 63

1. SLE
2. Sjogren's syndrome

Front 64

What disease is associated with a speckled ANA pattern and anti-Scl-70?

Back 64

Scleroderma

Front 65

What diseases are associated with a speckled ANA pattern and Anti-Jo/Mi2/PM/SRP?

Back 65

PM and DM

Front 66

Anti-Sm is indicative of which disease?

What type of ANA pattern would be demonstrated?

Back 66

SLE

Speckled

Front 67

What type of ANA pattern would Anti-Ro/La demonstrate?

Back 67

Speckled

(Anti-extractable nuclear antigens)

Front 68

What type of ANA pattern would Anti-Scl-70 demonstrate?

Back 68

Speckled

Front 69

What type of ANA pattern would anti-histone demonstrate?

Back 69

diffuse/homogenous

Front 70

What type of ANA pattern would Anti-RNP demonstrate?

Back 70

Speckled

Front 71

What type of pattern would Anti-dsDNA demonstrate?

Back 71

Peripheral (rim)

**SLE

Front 72

Autoantibody directed against the Fc portion of IgG.

Back 72

Rheumatoid factor

(RF is typically an IgM, but can be IgG or IgA).

Front 73

T or F.

The percentage of RF present in an individual increases with age.

Back 73

TRUE.

Front 74

Is RF specific for Rheumatoid arthritis?

Back 74

NO.

Not specific for RA and not sensitive to establish diagnosis

*However, it is present in 80% of patients with RA.

Front 75

Is Rheumatoid factor often detected in patients with Sjogren's syndrome?

Back 75

Yes. (75-95% of cases)

Front 76

Which autoantibody is highly specific and moderately sensitive for RA?

Back 76

Anti-CCP

(Can be detected many years before the diagnosis of RA)

Front 77

If you suspect an autoimmune condition based on the history, physical examination, and complete blood count, what test should you order?

Back 77

ANA test

Front 78

Which autoantibody is a defining feature of Mixed Connective Tissue Disease (MCTD)?

Back 78

Anti-RNP

Front 79

An uncommon autoimmune disorder that causes overlapping features of primarily three connective tissue diseases — lupus, scleroderma and polymyositis

Back 79

Mixed Connective Tissue Disease (MCTD)

Front 80

Which disease characteristically results in dry eyes, dry mouth, and dental caries?

Back 80

Sjogren's syndrome