Speckled Pattern ANA target Antigens And Associated Diseases

Question 0

RANA

rheumatoid associated nuclear antigen

Answer 0

RA

Question 1

Centromere (CENP)

Answer 1

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

PBC

Question 2

Jo-1

Which disease and which phenotype?

Answer 2

Polydermatomyositis

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

Question 4

Mi-2

Answer 4

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

Question 5

RNP

ribonucleoprotein

Answer 5

MCTD (100%)

Specificity 84-100%

Question 6

scl-70
Aka
Topoisomerase 1

Answer 6

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

Question 7

Sm

Smith antigen

Answer 7

SLE 30% sens

98% specific

Question 8

SS-A
aka
Ro

Answer 8

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)

Question 8

Pm-SCL

Answer 8

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

Question 9

SS-B
Aka
La

Answer 9

Sjogrens 60%
SLE 15%
rarely in other CTD

Not usually positive without ss-A

Question 11

Anti ribosomal P protein antibodies

Answer 11

High specificity low sensitivity (10-20%) SLE

Associated with neuropsychiatric lupus

Question 12

Anti RNP (anti ribonuclear protein)

Answer 12

95%-100% sensitivity MCTD

May also be high in SLE or Sjogrens

Question 13

Sensitivity and specificity of ANA in SLE

Answer 13

Sensitivity: 95%
Specificity: 55%

Question 14

Sensitivity and specificity of ANA in scleroderma

Answer 14

Sensitivity: 85%
Specificity: 55%

Question 15

Sensitivity and specificity of ANA in polymyositits/dermatomyositis

Answer 15

Sensitivity 60%

Specificity 65%

Question 16

Sensitivity and specificity of ANA in RA

Answer 16

Sensitivity 40%

Specificity 55%

Question 17

Sensitivity and specificity of ANA in Sjogren’s

Answer 17

Sensitivity 50%

Specificity 50%

Question 18

Use of ANA check in patient with Raynaud’s?

Answer 18

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

Question 19

DNA- histone complex (nucleosome) associations

Answer 19

SLE (60%)

Drug induced lupus (95%)

Question 20

ds DNA

Answer 20

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.

Question 21

RNA polymerase types II and III

Answer 21

systemic sclerosis- low sens but highly specific

Question 22

RNA polymerase 1, nucleolar RNA

Answer 22

systemic sclerosis- low sens but highly specific

Question 23

Pm- scl

Answer 23

polymyositis/scleroderma overlap syndrome

Question 24

anti- Th/To

Answer 24

Infrequent but specific for systemic sclerosis

Question 25

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

Answer 25

``` Jo1 PL-7 PL-12 EJ OJ ```

Question 26

Rheumatoid factor

Answer 26

``` 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 ```

Question 27

Sensitivity and specificity of RF in RA

Answer 27

Sensitivity 50-80% | Specificity 85-90% in advanced disease

Question 28

Sensitivity and specificity of anti-CCP in RA

Answer 28

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

Question 29

anti-Hu antibodies

Answer 29

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

Question 30

anti Ma-2 antibodies

Answer 30

limbic encephalitis associated with testicular cancer respond well to immunosupressive therapy

Question 31

anti-CRMP5

Answer 31

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

Question 32

anti NMDA receptor ab

Answer 32

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.

Question 33

anti-LGI1 antibodies

Answer 33

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

Question 34

Anti-SRP

Answer 34

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

Question 35

How do you interpret Complement testing?

Answer 35

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

Question 36

Which is better in Lupus flares? CRP or ESR?

Answer 36

ESR | CRP is often normal in flares

Question 37

HLAB27 associations?

Answer 37

``` Ank spond (strongest) Reactive arthritis Enteropathic spondyloarthritis Psoriatic Anterior uveitis Aortic incompetence with AV block ``` Normal in 5-8% caucasians

Question 38

What is the role of HLAB27 testing in ank spond?

Answer 38

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

Question 39

Scl-70

Answer 39

systemic sclerosis 40% in dcSSc 15% lcSSC Increased risk pulmonary involvement but NOT severity Reduced risk of renal involvement

Question 40

RNA polymerase

Answer 40

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

Question 41

Th/To

Answer 41

Poor outcome and pulmonary hypertension in lcSSc

Question 42

PM-SCl

Answer 42

myositis scleroderma overlap

Question 43

Centromere

Answer 43

60% limited cutaneous systemic sclerosis | less severe, better prognosis