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Y2 MSK > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

Systemic lupus ertythematosus is a CTD. What is the general pathology behind connective tissue disease?

A

Autoimmunity; antibodies produced against cells of the body;

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2
Q

SLE affects males more than females. True/False?

A

False

Females 9:1 males

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3
Q

Prevalence of SLE is higher in which ethnic groups?

A

Afro-Americans
Afro-Caribbeans
Asians

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4
Q

List general symptoms + signs of SLE

A 
Fatigue
Skin changes
Malaise
Joint pain
Alopecia
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5
Q

List skin changes in SLE

A

Malar/butterfly/discoid rash
Photosensitivity
N.B. sparing of nasolabial fold

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6
Q

SLE can be a totally cutaenous disease. True/False?

A

True

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7
Q

List musculoskeletal changes in SLE

A

Joint pain
Non-deforming arthritis
Myopathy
Raynauds

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8
Q

What is the difference between mild, moderate and severe SLE?

A

Mild - ‘simple’ cutaneous changes
Mod - arthritis, effusions etc.
Sev - organ-threatening disease

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9
Q

What are the main screening tests for SLE?

A

FBC
Urinalysis
Renal function
Antibodies

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10
Q

What are the main antibodies that are raised in SLE?

A 
ANA
Anti-dsDNA
Anti-Sm
Anti-Ro
Anti-La
Anti-RNP
Anti-PLD
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11
Q

ANA is the most useful/diagnostic antibody in SLE. True/False?

A

False

Anti-double-stranded DNA antibody is most specific

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12
Q

List general treatment for SLE

A

NSAIDs, analgesia
Anti-malarial
Steroids

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13
Q

What is the anti-malarial used for SLE?

A

Hydrochloroquine

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14
Q

Steroids can be used as monotherapy in SLE. True/False?

A

False

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15
Q

What is antiphospholipid syndrome?

A

Autoimmune disease where antibodies are produced against phospholipid

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16
Q

One lab and one clinical finding are required to diagnose antiphospholipid syndrome. List the potential lab findings

A

+ve antiphospholipid antibody
Lupus anticoagulation activity
anti-beta2-glycoprotein

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17
Q

One lab and one clinical finding are required to diagnose antiphospholipid syndrome. List the potential clinical findings

A

Thrombosis
Recurrent miscarriage
Levido reticularis rash

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18
Q

What is Sjogren’s syndrome?

A

Autoimmune destruction of exocrine glands

19
Q

What is systemic sclerosis?

A

Autoimmune inflammation and thickening of skin due to increased collagen deposition leading to fibrosis and vascular endothelial changes

20
Q

Raynaud’s phenomenon is usually present in systemic sclerosis - what happens?

A

Reduced blood flow/vasculopathy

21
Q

Limited systemic sclerosis was known as CREST - what does this stand for?

A 
Calconosis
Raynaud's
Esophageal dysmotility
Sclerodactyly
Telangectasia
22
Q

Which antibodies are associated with systemic sclerosis?

A

Anti-centromere (limited)

Anti-Scl-70 (diffuse)

23
Q

Which antibody is associated with Sjogren’s syndrome?

A

Anti-Ro

Anti-La

24
Q

SLE is more common in what age range?

A

20s - 40s

25
Q

Complement levels are high in active SLE. True/False?

A

False

Low

26
Q

Why is urinalysis done in SLE?

A

Look for glomerulonephritis

27
Q

Outline management for mild (skin) SLE

A

Hydroxychloroquine
Topical steroid
NSAID

28
Q

Outline management for moderate SLE

A

Immunosuppression (azathioprine, methotrexate)

Oral steroid

29
Q

Outline management for severe SLE

A

IV steroid
Cyclophosphamide
Rituximab

30
Q

List clinical features of Sjogren’s syndrome

A

Dry mouth/eyes (sicca)
Parotid swelling
Fatigue

31
Q

What is the difference between limited and diffuse systemic sclerosis?

A

Limited - mainly confined skin changes

Diffuse - rapid skin changes and early organ involvement

32
Q

List features of mixed CTD

A 
Raynaud's phenomenon
Arthralgia/arthritis
Myositis
Sclerodactyly
Pulmonary hypertension
ILD
33
Q

What does antiphospholipid syndrome increase the risk of?

A

Increased clotting, leading to thrombosis

34
Q

In females, what is the important common consequence of antiphospholipid syndrome (APS)?

A

Pregnancy loss

35
Q

Outline treatment for APS

A

Anticoagulation for those with thrombosis

LMWH in pregnancy

36
Q

List aetiology of SLE

A

Harmonal (increased oestrogen exposure e.g. contraceptive and HRT, early menarche)
Environmental (viruses - EBV, UV light, silica dust)

37
Q

What is the basic pathogenesis of SLE?

A

Increased and defective apoptosis

38
Q

What investigations are carried out do diagnose Sjogren’s syndrome?

A

Labial gland biopsy

Shirmers test

39
Q

Outline the management of Sjogren’s syndrome

A

Eye drops
Saliva supplements
Hydroxychloroquinine
Immunosuppresants

40
Q

What class of drug is used to treat Raynaud’s phenomenon?

A

Ca channel blocker

41
Q

Which auto-antibody is associated with mixed CTD?

A

Anti-RNP

42
Q

Which HLA allele is susceptible in SLE?

A

HLA-DR3

43
Q

Which type of hypersensitivity reaction is involved in SLE?

A

Type III

Y2 MSK (30 decks)

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