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MyDermatology-Bolognia > Ch. 40-Autoantibodies in rheumatic disease > Flashcards

Ch. 40-Autoantibodies in rheumatic disease Flashcards

1
Q

Most sensitive Ab in lupus

A

ANA

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2
Q

Most specific 3 antibodies in lupus

A

anti-dsDNA*
Anti-Sm*
rRNP (sometimes called just RNP/U1RNP-seen in MCTD)

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3
Q

Drug induced SLE? What %?

A

anti-histone

95%

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4
Q

What antibodies for disease activity

A

c3/c4

dsDNA

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5
Q

What % patients SLE + aNA

A

99%

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6
Q

What % patients dsDNA

A

60%

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7
Q

What is dsDNA associated with?

A

lupus nephritis

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8
Q

What % SLE + for anti smith

A

10-30% cause
30-40 asian and African American

30%

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9
Q

What % SLE + RNP

A

7-15%, 40% asians

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10
Q

RNP association

A

neuropsychiatric

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11
Q

What ANA pattern associated with SLE

A

Homogenous
Speckled
Peripheral

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12
Q

Name 10 antibodies seen in SLE

A

Specific:
dsDNA-60
Sm-30
RNP-30

B2 glycoprotein-25%
cardiolipin-50%

Less specific
ANA-99
ssDNA -70%
c1Q-60%
Histone-40 %
SSA-50%
U1RNP-50%
PCNA-50%
ku
SSB
RF
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13
Q

What Ab is neonatal lupus associated with?

A

Anti-SSA/ro

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14
Q

What ab SCLE associated with?

A

SSA/ro

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15
Q

What Ab is drug induced SCLE associated with?

A

SSA/Ro

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16
Q

What ab associated with possible risk for SLE in DLE patients;

A

ssDNA

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17
Q

What is c1Q associated with

A

Hypocomplementemic urticarial vasculitis and severe SLE

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18
Q

What is mixed CTD

A

Lupus
Scleroderma
Polymyositis

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19
Q

What Ab associated with Mixed CTD? what % have it?

A

Anti-U1RNP (RNP sometimes called)

100%

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20
Q

What % DM ANA +

A

40%

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21
Q

What are the DM specific antibodies

A 
Mi-2
Jo-1 (PL-7, PL-12, OJ, EJ)
TIFF/P155
MDA5
NXP2
SAE1
SRP
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22
Q

What Ab’s in APLS

A

Anticardiolipin
B2-glycoprotein

lupus inhibitor/anticoagulant

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23
Q

True or false: Amyopathic DM is more associated with ANA + than myopathic?

A

True! Present in 65% amyopathic DM

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24
Q

Which 2 DM antibodies are clinically amyopathic

A

Tiff-1 gamma/p155
and
MDA5

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25
Q

What is DM with MI-2 + associated with? What % DM are mi-2 +?

A

15%

Myositis with proximal muscle weakness

Skin: Classic cutaneous DM

No malignancy association

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26
Q

What is features of DM with Jo-1 positivity? What %?

A

20%

Anti-synthetase syndrome

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27
Q

What Ab’s associated with anti-sdnthetase

A 
Jo-1*
PL-7
PL-12
OJ
EJ
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28
Q

Does mechanics. hands occur in only anti-synthetase syndrome?

A

No. Occurs in amyopathic variants as well.

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29
Q

What is anti-sythetase syndrome

A

Mechanics hands
ILD
Raynauds
Arthritis

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30
Q

What is the other name for P155

A

TIF-1 gamma

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31
Q

What % DM patients are p155/TIFF-1 positive?

A

20–30% classic DM

80% of those who are amyopathic

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32
Q

Features of P155/TIFF-1 gamma DM?

A
  • Clinically amyopathic 80% of the time
  • highest risk malignancy

Skin:

  • diffuse photo distributed erythema
  • palatal erythema (“ovoid patches”)
  • psoriasiform lesions
  • keratotic palmar papules
  • hypopigmented and telangiectatic (“red on white”) patches
  • infrequent calcinosis
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33
Q

Anti-SRP features DM?

A

Fulminant DM/PM with cardiac involvement
Drug resistant PM
High mortality rate

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34
Q

DM associated with MDA-5/CADM-140 features

A

Clinically amyopathic DM

Rapidly progressive interstitial lung disease ( most common in asian)–> can use this ab to monitor progression

Cutaneous:

  • oral pain and ulceration
  • cutaneous ulcerations with associated vasculopathy (mostly on fingers)
  • tender palmar papules
  • hand swelling and arthritis/arthralgia
  • diffuse hair loss

Can be malignancy associated

35
Q

NXP-2 DM associations?

A

Adults:

  • 2nd highest risk malignancy
  • subcutaneous dema
  • calcinosis cutis

In juveniles:
- associated with more severe muscle disease and calcinosis

36
Q

DM ab’s associated with highest risk malignancy?

A

TIFF-1/P155 > NXP-2

37
Q

Anti-SAE1 associated DM?

A

Dysphagia
Diffuse cutaneous disease with dark red diffuse skin rash (particularly in asian)
100% have rash, in addition to typical find gins
Psoriasiform reaction to plaquenil

38
Q

What are the DM “associated” antibodies

A

ANA (speckled, nucleolar) 40%
ssDNA 40%
There are some others..

39
Q

What 3 antibodies are associated with systemic sclerosis

A

Anti-centromere
Anti-SCL70 (topoisomerase I)
Anti-RNA polymerase III (RNAP)

40
Q

What ab associated with limited systemic sclerosis (CREST syndrome)? What %?

A

Anti-centremore

80+ %

41
Q

What Scleroderma Ab associated with increased risk malignancy and may indicate paraneoplastic scleroderma?

A

anti-RNA polymerase III

42
Q

Most prevalent Ab in scleroderma

A

ANA

43
Q

What 2 Ab associated with diffuse systemic sclerosis?

A

DNA topoisomerase

RNP polymerase

44
Q

What antibodies overlap between morphea and systemic sclerosis

A 
ANA (40%-more observed in patients with widespread disease) vs. 95% systemic sclerosis
ssDNA ( most in linear morphea)
Fibrillar
Histone
RF

Present in 30-50% morphea

NO SCL-70/RNP/Cm

45
Q

What Ab’s associated with Sjogrens and what prevalence?

A

Alpha fodrin-70%
SSA/ro-60%
SSB/la-20%

46
Q

What % population has ANA 1:80?

A

10-15% at 1 : 80
5% at 1 : 160
3% at 1 : 320

47
Q

What are 3 reasons someone may have elevated ANA without an AI-CTD?

A

the elderly

relatives of those with SLE, and patients with other autoimmune disorders (e.g. autoimmune thyroid disease),

48
Q

Which ANA pattern are associated out with SLE

A

Homogenous
Speckled
Peripheral/rim-dsDNA

49
Q

Differences between ANA screen and Hep-2?

A

ANA screen is an ELISA–>cheaper/easier, higher rate false positives

Hep-2 is indirect IF on Hep-2 cells–> More sensitive but more expensive

50
Q

Why is Hep-2 more sensitive?

A

Larger array and higher concentration of nuclear antigens -e.g.

  • SSA
  • centromere antigens

ANA screen = 8 to 10 autoantigens
Hep2= with >100 autoantigens in HEp-2 cells.

51
Q

What does CRP increase in response to, specifically

A

IL-6

52
Q

What are the 6 antibodies in an ENA test?

A 
Ro
La
Sm
RNP
Scl-70
Jo1
53
Q

What do we deem + ANA?

A

1:160

54
Q

Methods of ANA screen vs. Hep-2?

A

ANA screen is ELISA: plate with 8-10 antigens

Hep-2 is indirect immunofluorescence on human cell line

55
Q

True or false: Following ANA titres is good for disease monitoring?

A

False, can take 7 mo to change and doesn’t typically correlate

56
Q

Other than lupus, what 3 AI-CTD are most associated with ANA +

A

Systemic sclerosis-90%
sjogrens 70%
DM/PM 40-65%

57
Q

Work up for suspected lupus patient with ANA >1:160

A 
Autoab: dsDNA, cardiolipnin/b2GP, SSA/SSB, sm
Other: 
CBC with diff
Albumin, Cr, total protein 
PT/PTT
LEs
UA + PCR
ESR/CRP
C3/C4
58
Q

What markers to follow for SLE activity

A

dsDNA
complements

*DSDNA esp. for lupus nephritis

59
Q

What marker to follow for GPA?

A

c-ANCA

60
Q

What ab is high risk for scleroderma renal crisis ?

A

Anti-RNA polymerase

61
Q

2 lupus abs in ACR criteria

A

Sm
dsDNA

Specific!

62
Q

Why are SSA and SSB often both positive

A

linked set of auto antigens present in same set RNP particles, thus develops against one and spread to the other.

*cavaeta that SSA can occur alone, but SSB rarely occurs alone

63
Q

What are the RNP auto’s (ribonucleic proteins)

A

SSA (hyRNP) (SLE, SCLE, Sjögrens, neonatal lupus, SCLE-sjogrens overlap
SSB (hyRNP) (SCLE, sCLE-sjogrens overlap, primary sjogrens)
rRNP-SLE
U1RNP-MCTD
anti Smith

64
Q

SCLE main ab? what % have it?

A

SSA

75-90%

65
Q

Sx drug induced SLE?

A

MSK- arthritis, arthralgia, myalgia
Serositis

Much less often skin

66
Q

Anti-TNF induced SLE associated with which ab’s?

A

ANA and anti-dsDNA

67
Q

Which anti-tinf least likely to induce lupus like syndrome

A

etanercept

68
Q

In patients on anti-tnfs, seroconversion to + ANA and anti-dsDNA was associated with what?

A

Formation of anti-drug antibodies with loss of clinical response

69
Q

What is different about anti-tnf induced lupus vs. SLE

A

Rare kidney involvement

70
Q

2 Ab’s associated with RA and prevalence of those Ab’s?

A

RF-80%

CCP-70%

71
Q

What is a RF

A

Ab directed against the FC portion of IgG

72
Q

Most specific ab for Sjogrens

A

Alpha-fodrin-70%

73
Q

Where is alpha-fodrin ab founds

A

Sjogrens

SLE

74
Q

P-ANCA associated with which vasculitis

A

EGPA

MPA

75
Q

C-ANCA associated with which ANCA vasculitis

A

GPA

76
Q

C-ANCA primarily targets what?

A

PR3-antigens in cytoplasm of neutrophils

77
Q

What is an ANCA

A

Anti-neutrophilic cytoplasmic antibodies

Antibodies directed against cytoplasmic granules of neutrophils

Can be cytoplasmic (C-ANCA) or peri-nuclear (P-ANCA)

Staining for these shows either cytoplasmic pattern =C-ANCA or perinculear pattern

78
Q

What antigen does P-anca predominately target

A

MPO-myeloperoxidase

79
Q

Which is more specific: C-ANCA for PR3 for P-ANCA for MPO

A

C-ANCA for PR3 (sometimes is due to MPO or BPI but rarely)

P-ANCA can detect MPO, lactoferrin, elastase, BPI, cathepsin G

80
Q

What Ab’s found in GPA and how often?

A

PR3 in 90%

MPO+ up to 50% in asians (10% in non asians)

81
Q

What ab’s found in EGPA and how often?

A

P-ANCA
MPO+ in 30-40%

*Least associated of all 3

82
Q

What ab’s found in MPA

A

MPO+ 90%
P-ANCA

*PR3 in 20-30%

83
Q

What conditions (other than AAV) can you see ANCA +?

A

Most often it is P-ANCA than C-ANCA
*Some of these are PR3 and MPO related, some are not

  1. Infections
    - respiratory infections, HIV, parvo, subacute endocarditis (C!), enteritis, Hep-C
  2. AI CTD
    -lupus, RA, scleroderma, > sjogrens, DM
  3. Inflammatory GI
    -AI hepatitis (70%) and PSC (80%), PBC 30%
    -UC >chrons
  4. Drug induced vasculitis/lupus/hepatitis
    -PTU, minocycline, hydralazine, penicillamine TNF, IVIG
  5. Other GN/vasculitis
    -anti GBM, beeches, immune complex, post-strep, HSP, burgers,
    Other: Cystic fibrosis C-ANCA 80%

MyDermatology-Bolognia (31 decks)

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