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MSK > Connective Tissue Disorders > Flashcards

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
Q

Once SLE is diagnosed, what is the next step?

A

Investigate organ involvement with a range of tests (e.g. CXR, PFTs, echo, renal biopsy).

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26
Q

How is SLE monitored?

A

1) Anti-dsDNA which positively correlates with disease activity
2) C3/C4 levels which negatively correlate with disease activity

27
Q

What are the basic steps of SLE treatment?

A

1) Regular self-monitoring
2) Counselling
3) Avoid sun exposure

28
Q

How is “mild” SLE treated

A

NSAIDs & simple analgesia

29
Q

If an SLE patient develops arthritis or cutaneous lesions, which drugs are warranted?

A

Hydroxychloroquine, steroids (low dose, <15mg/day).

30
Q

What symptoms of SLE warrant high dose steroids (IV or 1mg/kg/day)?

A

Organ dysfunction or diffuse GN

31
Q

What symptoms of SLE warrant medium-dose steroids (0.5mg/kg/day)?

A

Resistant serositis

32
Q

Immunosuppressives should be used in which severity of SLE?

A

Moderate SLE

33
Q

Biologics should be used in which severity of SLE?

A

Severe (organ involvement)

34
Q

What is the antibody class in APS?

A

IgM or IgG

35
Q

How is APS managed? (3)

A

1) Lifelong anticoagulation
2) Aspirin/ heparin in pregnancy
3) Hydroxychloroquine

36
Q

What symptoms does Sjogren’s Syndrome cause

A

“Drying” (decreased lacrimal & salivary gland production): dry mouth, dry throat, vaginal dryness. Increased dental carries, bilateral salivary gland enlargement.

37
Q

What antibodies are positive in Sjogren’s Syndrome?

A

Anti-Ro and Anti-La (as you Ro(w) towards La(nd) you nearly touch your eyes)

38
Q

What non-antibody tests are used in Sjogren’s?

A

Salivary gland biopsy, labial biopsy

39
Q

How is Sjogren’s treated? (5)

A

Artificial tear drops, ciclosporin eye drops, punctal plugs, hydroxychloroquine, methotrexate

40
Q

What is the underlying cause of systemic sclerosis?

A

Recurrent autoimmunity leading to fibrosis and vascular endothelial changes

41
Q

Diffuse cutaneous systemic sclerosis usually involves which area?

A

Torso & skin proximal to forearm

42
Q

Diffuse cutaneous systemic sclerosis usually presents with early/late organ involvement?

A

Early (especially ILD). Can lead to renal crisis.

43
Q

Which antibodies are +ve in diffuse cutaneous systemic sclerosis? (3)

A

Anti-topioisomerase
Anti-Scl-70
Anti-RNA III

44
Q

What is limited SSc also known as?

A

CREST syndrome

45
Q

What are the symptoms of CREST/ limited SSc?

A 
Calcinosis
Raynauld's
Esophageal dysfunction
Sclerodactyly
Telangiectasia
46
Q

What’s a common complication of CREST/ SSc?

A

Pulmonary HT

47
Q

Which antibody is positive in CREST?

A

Anti-Centromere, Anti-Scl-70

48
Q

How is systemic sclerosis treated?

A 
Depends upon symptoms: 
Raynaulds: PDE inhibitor, calcium-channel blocker
Lung disease: Immunosuppression
Reflux: PPI
Tight BP control
Digital ulcers: iloprost infusion
49
Q

What antibody is positive in MCTD?

A

Anti-RNP

50
Q

What’s a common complication of MCTD?

A

Pulmonary hypertension

51
Q

Antibody positive in Sjogren’s

A

Anti-Ro, Anti-La

52
Q

Antibody positive in SSc

A

Anti-centromere, anti-Scl-70

53
Q

Antibody positive in diffuse SSc

A

Anti-topioisomerase, anti-RNA III

54
Q

Antibody positive in MCTD

A

Anti-RNP

55
Q

Antibody positive in Polymyositis

A

Anti-Jo-1

56
Q

Antibody positive in APS

A

Anti-Cardiolipin, Lupus Anticoagulant, anti-beta2 glycoprotein

57
Q

SLE is what type of HS reaction?

A

Type III

58
Q

Goodpasture’s Syndrome is what type of HS reaction?

A

Type II

59
Q

Farmer’s Lung is what type of HS reaction?

A

Type III

60
Q

Rheumatic fever can follow which infection

A

Streptococcus

61
Q

Mnemonic for SLE

A 
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
Q

Each individual has how many HLAs?

A

2 (type I and II)

63
Q

What is IPEX syndrome

A

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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