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?

25
Complement levels are high in active SLE. True/False?
False | Low
26
Why is urinalysis done in SLE?
Look for glomerulonephritis
27
Outline management for mild (skin) SLE
Hydroxychloroquine Topical steroid NSAID
28
Outline management for moderate SLE
Immunosuppression (azathioprine, methotrexate) | Oral steroid
29
Outline management for severe SLE
IV steroid Cyclophosphamide Rituximab
30
List clinical features of Sjogren's syndrome
Dry mouth/eyes (sicca) Parotid swelling Fatigue
31
What is the difference between limited and diffuse systemic sclerosis?
Limited - mainly confined skin changes | Diffuse - rapid skin changes and early organ involvement
32
List features of mixed CTD
``` Raynaud's phenomenon Arthralgia/arthritis Myositis Sclerodactyly Pulmonary hypertension ILD ```
33
What does antiphospholipid syndrome increase the risk of?
Increased clotting, leading to thrombosis
34
In females, what is the important common consequence of antiphospholipid syndrome (APS)?
Pregnancy loss
35
Outline treatment for APS
Anticoagulation for those with thrombosis | LMWH in pregnancy
36
List aetiology of SLE
Harmonal (increased oestrogen exposure e.g. contraceptive and HRT, early menarche) Environmental (viruses - EBV, UV light, silica dust)
37
What is the basic pathogenesis of SLE?
Increased and defective apoptosis
38
What investigations are carried out do diagnose Sjogren's syndrome?
Labial gland biopsy | Shirmers test
39
Outline the management of Sjogren's syndrome
Eye drops Saliva supplements Hydroxychloroquinine Immunosuppresants
40
What class of drug is used to treat Raynaud's phenomenon?
Ca channel blocker
41
Which auto-antibody is associated with mixed CTD?
Anti-RNP
42
Which HLA allele is susceptible in SLE?
HLA-DR3
43
Which type of hypersensitivity reaction is involved in SLE?
Type III