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Immunology > Speckled Pattern ANA target Antigens And Associated Diseases > Flashcards

Speckled Pattern ANA target Antigens And Associated Diseases Flashcards

0
Q

RANA

rheumatoid associated nuclear antigen

A

RA

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1
Q

Centromere (CENP)

A

limited cutaneous systemic sclerosis (sens 30%, spec 97%)

PBC

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2
Q

Jo-1

Which disease and which phenotype?

A

Polydermatomyositis

Associated with ILD, mechanics hands, and Raynaud’s phenomenon

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4
Q

Mi-2

A 
Dermatomyosotis
favourable long-term prognosis
Steroid responsive
"shawl sign"
less cancer
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5
Q

RNP

ribonucleoprotein

A

MCTD (100%)

Specificity 84-100%

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6
Q

scl-70
Aka
Topoisomerase 1

A

systemic sclerosis- positive in 20% and is highly specific (100%) for diffuse disease
RARE to coexist with centromere Ab

Good to predict pulmonary fibrosis and developing diffuse cutaneous involvement

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7
Q

Sm

Smith antigen

A

SLE 30% sens

98% specific

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8
Q

SS-A
aka
Ro

A

Primary sjogrens 75% sensitive
SLE 35%
Secondary sjogren’s 15%
Presence is associated with developing extraglandular features in Sjogren’s (vasculitis, lymphadenopathy, nephritis, leukopaenia, photosensitivity, subacute cutaenous lupus, neonatal lupus and congenital heart block)

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8
Q

Pm-SCL

A

5% scleroderma alone
8% polymyositis alone
25% polymyositis/scleroderma overlap syndrome

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9
Q

SS-B
Aka
La

A

Sjogrens 60%
SLE 15%
rarely in other CTD

Not usually positive without ss-A

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11
Q

Anti ribosomal P protein antibodies

A

High specificity low sensitivity (10-20%) SLE

Associated with neuropsychiatric lupus

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12
Q

Anti RNP (anti ribonuclear protein)

A

95%-100% sensitivity MCTD

May also be high in SLE or Sjogrens

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13
Q

Sensitivity and specificity of ANA in SLE

A

Sensitivity: 95%
Specificity: 55%

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14
Q

Sensitivity and specificity of ANA in scleroderma

A

Sensitivity: 85%
Specificity: 55%

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15
Q

Sensitivity and specificity of ANA in polymyositits/dermatomyositis

A

Sensitivity 60%

Specificity 65%

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16
Q

Sensitivity and specificity of ANA in RA

A

Sensitivity 40%

Specificity 55%

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17
Q

Sensitivity and specificity of ANA in Sjogren’s

A

Sensitivity 50%

Specificity 50%

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18
Q

Use of ANA check in patient with Raynaud’s?

A

Estimate risk of developing an associated systemic rheumatic disease (19-30% with positive ANA, compared with 7% without ANA)

19
Q

DNA- histone complex (nucleosome) associations

A

SLE (60%)

Drug induced lupus (95%)

20
Q

ds DNA

A

SLE
57% sensitive
97% specific
present at some time in the course of the disease but fluctuate and may be absent
Also can be seen in RA, Sjogren’s, scleroderma, drug induced lupus, Raynaud’s, MCTD, myositis, chronic active hepatitis, uveitis, Grave’s, and in women with silicone breast implants.
Unless very high level of suspicion, do not check unless ANA elevated.

21
Q

RNA polymerase types II and III

A

systemic sclerosis- low sens but highly specific

22
Q

RNA polymerase 1, nucleolar RNA

A

systemic sclerosis- low sens but highly specific

23
Q

Pm- scl

A

polymyositis/scleroderma overlap syndrome

24
Q

anti- Th/To

A

Infrequent but specific for systemic sclerosis

25
Q

List the autoantibodies against a group of aminoacyl tRNA synthetases in dermatomyositis and polymyositis

A 
Jo1
PL-7
PL-12
EJ
OJ
26
Q

Rheumatoid factor

A 
RA
Sjogren's
SLE
systemic sclerosis
Cryoglobulinaemia
Dermatomyositis, polymyositis
SBE
Malignancy
Infection (hepatitis, leprosy, parasites, syphilis, TB)
Pulmonary disease (ALD, sarcoid, silicosis)
Primary biliary cirrhosis
27
Q

Sensitivity and specificity of RF in RA

A

Sensitivity 50-80%

Specificity 85-90% in advanced disease

28
Q

Sensitivity and specificity of anti-CCP in RA

A

Sensistivity 30-60%
Specificity 95-98%
May be positive before disease emerges
May predict more rapid radiological progression

29
Q

anti-Hu antibodies

A

limbic or brainstem encephalitis- small cell lung ca found in all of these if anti-Hu ab present.
Can also cause a subacute sensory neuropathy by attacking the DRG

30
Q

anti Ma-2 antibodies

A

limbic encephalitis
associated with testicular cancer
respond well to immunosupressive therapy

31
Q

anti-CRMP5

A

anti-CRMP5 encephalomyelitis
most common association is with SCLC and thymoma
cortical symptoms not often confined to the limbic system

32
Q

anti NMDA receptor ab

A

anti NMDA receptor encephalitis
classical syndrome: psych manifestations, insomnia, memory deficits, seizures, dyskinesias, autonomic instability, language dysfunction including echolalia
Half of girls have an underlying ovarian teratoma. May not be associated with a tumour.

33
Q

anti-LGI1 antibodies

A

not actually against voltage gated potassium channels as previously thought. Instead secreted neural protein that is a ligand for ADAM22 and 23 which are epilepsy proteins.
Memory disturbance, confusion, faciobrachial dystonic seizures, hyponatraemia

34
Q

Anti-SRP

A

An ENA in necrotising myopathy
confers poor prognosis
Called Signal recognition protein

35
Q

How do you interpret Complement testing?

A

Both low–>disease activity in lupus. Also 10% lupus patients are C4 deficient.
C4 lower than C3–>cryoglobulinaemia
Isolated C3 depression–>seen in membranoproliferative GN

36
Q

Which is better in Lupus flares? CRP or ESR?

A

ESR

CRP is often normal in flares

37
Q

HLAB27 associations?

A 
Ank spond (strongest)
Reactive arthritis
Enteropathic spondyloarthritis
Psoriatic
Anterior uveitis
Aortic incompetence with AV block

Normal in 5-8% caucasians

38
Q

What is the role of HLAB27 testing in ank spond?

A

90% positive
inflammatory back pain in under 40s
absence of radiological changes eg early disease
Pos likelihood ratio 15

39
Q

Scl-70

A

systemic sclerosis
40% in dcSSc
15% lcSSC

Increased risk pulmonary involvement but NOT severity
Reduced risk of renal involvement

40
Q

RNA polymerase

A

Seen in 20% of systemic sclerosis
Associated with renal disease or severe skin disease
Can be used to assess risk of renal crisis in patients with early diffuse disease

41
Q

Th/To

A

Poor outcome and pulmonary hypertension in lcSSc

42
Q

PM-SCl

A

myositis scleroderma overlap

43
Q

Centromere

A

60% limited cutaneous systemic sclerosis

less severe, better prognosis

Immunology (16 decks)

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