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Connective Tissue Disorders Flashcards

1
Q

SLE is more common in which sex?

A

Females

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2
Q

SLE is commoner in which ethnic groups

A

Asians, Afro-Caribbeans

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3
Q

What is a genetic trigger for SLE?

A

Oestrogen exposure (e.g. HRT, early menarche)

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4
Q

What is an environmental trigger for SLE?

A

EBV infection

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5
Q

Is SLE B / T-cell response?

A

Both

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6
Q

In SLE, what is the target?

A

Nuclear material

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7
Q

How many SLE diagnosis criteria need to be met before SLE can be diagnosed?

A

More than or equal to 4 (at least 1 lab and 1 clinical)

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8
Q

What are some clinical diagnostic signs of SLE?

A

Cutaneous lupus, oral ulcers, arthritis

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9
Q

What are some laboratory diagnostic signs of SLE?

A

ANA, Anti-DNA, anti-Sm

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10
Q

SLE general symptoms

A

Fever, malaise, poor appetite, weight loss

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11
Q

Mucocutaneous symptoms of SLE include

A

Photosensitive rash, malar rash, painless ulcers on mouth, alopecia

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12
Q

MSK symptoms of SLE include

A

Deforming arthropathy “Jaccoud’s arthritis”, non-erosive arthritis, myopathy

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13
Q

Serositis symptoms of SLE

A

Pericarditis

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14
Q

Renal symptoms of SLE

A

Proteinuria (>500mg/day)

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15
Q

CNS symptoms of SLE

A

Depression, headache

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16
Q

Haematological symptoms of SLE

A

Lymphadenopathy (25% of patients), thrombocytopenia, leucopenia

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17
Q

What are some clinical symptoms/ signs for anti-phospholipid syndrome?

A

Recurrent venous & arterial thrombosis (esp. in young patients), recurrent miscarriage (esp. <10 weeks), livido reticularis, prolonged APTT

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18
Q

Livido reticularis is associated with which condition?

A

APS

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19
Q

How useful is an ANA in SLE diagnosis?

A

Limited - it will be positive in 95% of SLE patients but also positive in 20% of healthy people. It is also associated with HIV and HCV infection.

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20
Q

How useful is anti-dsDNA in SLE diagnosis?

A

Very specific for SLE. Titre correlates with disease activity

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21
Q

Is Anti-Ro positive in SLE?

A

Yes (also in Sjogren’s)

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22
Q

Is anti-Sm positive in SLE?

A

Highly specific for SLE. If positive suggests CNS involvement.

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23
Q

What are the APS antibodies?

A

Cardiolipin, lupus anticoagulant, anti-beta2 glycoprotein

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24
Q

What criteria need to be met for a diagnosis of APS to be made?

A

1) Positive anti-cardiolipin / lupus anticoagulant / anti-beta2 glycoprotein
2) Need to be positive on at least 2 occasions 12 weeks apart

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25
Once SLE is diagnosed, what is the next step?
Investigate organ involvement with a range of tests (e.g. CXR, PFTs, echo, renal biopsy).
26
How is SLE monitored?
1) Anti-dsDNA which positively correlates with disease activity 2) C3/C4 levels which negatively correlate with disease activity
27
What are the basic steps of SLE treatment?
1) Regular self-monitoring 2) Counselling 3) Avoid sun exposure
28
How is "mild" SLE treated
NSAIDs & simple analgesia
29
If an SLE patient develops arthritis or cutaneous lesions, which drugs are warranted?
Hydroxychloroquine, steroids (low dose, <15mg/day).
30
What symptoms of SLE warrant high dose steroids (IV or 1mg/kg/day)?
Organ dysfunction or diffuse GN
31
What symptoms of SLE warrant medium-dose steroids (0.5mg/kg/day)?
Resistant serositis
32
Immunosuppressives should be used in which severity of SLE?
Moderate SLE
33
Biologics should be used in which severity of SLE?
Severe (organ involvement)
34
What is the antibody class in APS?
IgM or IgG
35
How is APS managed? (3)
1) Lifelong anticoagulation 2) Aspirin/ heparin in pregnancy 3) Hydroxychloroquine
36
What symptoms does Sjogren's Syndrome cause
"Drying" (decreased lacrimal & salivary gland production): dry mouth, dry throat, vaginal dryness. Increased dental carries, bilateral salivary gland enlargement.
37
What antibodies are positive in Sjogren's Syndrome?
Anti-Ro and Anti-La (as you Ro(w) towards La(nd) you nearly touch your eyes)
38
What non-antibody tests are used in Sjogren's?
Salivary gland biopsy, labial biopsy
39
How is Sjogren's treated? (5)
Artificial tear drops, ciclosporin eye drops, punctal plugs, hydroxychloroquine, methotrexate
40
What is the underlying cause of systemic sclerosis?
Recurrent autoimmunity leading to fibrosis and vascular endothelial changes
41
Diffuse cutaneous systemic sclerosis usually involves which area?
Torso & skin proximal to forearm
42
Diffuse cutaneous systemic sclerosis usually presents with early/late organ involvement?
Early (especially ILD). Can lead to renal crisis.
43
Which antibodies are +ve in diffuse cutaneous systemic sclerosis? (3)
Anti-topioisomerase Anti-Scl-70 Anti-RNA III
44
What is limited SSc also known as?
CREST syndrome
45
What are the symptoms of CREST/ limited SSc?
``` Calcinosis Raynauld's Esophageal dysfunction Sclerodactyly Telangiectasia ```
46
What's a common complication of CREST/ SSc?
Pulmonary HT
47
Which antibody is positive in CREST?
Anti-Centromere, Anti-Scl-70
48
How is systemic sclerosis treated?
``` Depends upon symptoms: Raynaulds: PDE inhibitor, calcium-channel blocker Lung disease: Immunosuppression Reflux: PPI Tight BP control Digital ulcers: iloprost infusion ```
49
What antibody is positive in MCTD?
Anti-RNP
50
What's a common complication of MCTD?
Pulmonary hypertension
51
Antibody positive in Sjogren's
Anti-Ro, Anti-La
52
Antibody positive in SSc
Anti-centromere, anti-Scl-70
53
Antibody positive in diffuse SSc
Anti-topioisomerase, anti-RNA III
54
Antibody positive in MCTD
Anti-RNP
55
Antibody positive in Polymyositis
Anti-Jo-1
56
Antibody positive in APS
Anti-Cardiolipin, Lupus Anticoagulant, anti-beta2 glycoprotein
57
SLE is what type of HS reaction?
Type III
58
Goodpasture's Syndrome is what type of HS reaction?
Type II
59
Farmer's Lung is what type of HS reaction?
Type III
60
Rheumatic fever can follow which infection
Streptococcus
61
Mnemonic for SLE
``` SOAP-BRAIN-MD. S: serotitis O: Oral ulcers A: Arthritis P: Photosensitivity B: Blood (all low; anaemia, thrombocytopaenia) R: Renal (protein A: ANA +ve I: Immunology (anti-dsDNA) N: Neurologic (psych, seizures, depression) M: Malar rash D: Discoid rash ```
62
Each individual has how many HLAs?
2 (type I and II)
63
What is IPEX syndrome
A monogenic cause of severe autoimmunity due to mutation in FOXP3 gene required to induce T-regulatory cell production

MSK (21 decks)

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