Connective Tissue Diseases

Question 1

These are diseases of connective tissue. True or false?

Answer 1

False

Question 2

SLE is what type of disease?

Answer 2

Autoimmune

Question 3

What type of hypersensitivity disease is SLE?

Answer 3

Type III- immune complex mediated

Question 4

Normally cells break down and are cleared by the immune system. Is this process faster or slower in SLE?

Answer 4

Slower

Question 5

What are antibodies formed against in SLE?

Answer 5

Contents of broken down cells which have not been cleared due to defective immune system

Question 6

Which sex is SLE more common in?

Answer 6

Females

Question 7

What race is SLE more common in?

Answer 7

Asians

Question 8

What is the extent of SLE generally in males?

Answer 8

They are less likely to develop it, but if they do the extent is usually much worse

Question 9

Is there a genetic component to SLE?

Answer 9

Yes

Question 10

Which hormone is related to SLE?

Answer 10

Oestrogen

Question 11

What are 3 examples of environmental factors which may influence SLE?

Answer 11

Viruses, silica dust and UV light

Question 12

Is apoptosis increased or decreased in SLE?

Answer 12

Increased

Question 13

What is the most important thing to look out for in patients with either diagnosed or potential SLE? Why?

Answer 13

Renal disease- it causes no clinical signs or symptoms

Question 14

Where do immune complexes get deposited in the kidneys in SLE?

Answer 14

Mesangium

Question 15

What is the mesangium?

Answer 15

A structure associated with capillaries in the glomerulus

Question 16

Where do immune complexes form in SLE?

Answer 16

In the circulation

Question 17

What is serositis?

Answer 17

Inflammation of the lining of something

Question 18

What are some mucocutaneous features of SLE?

Answer 18

• Photosensitivity
• Malar rash
• Discoid lupus
• Mouth ulcers
• Non- scarring alopecia

Question 19

Where does the malar rash of SLE usually spare?

Answer 19

Naso-labial folds

Question 20

A lupus rash only lasts for a short time. True or false?

Answer 20

False- it will last for a long time after it has started

Question 21

In terms of the hands, what is the difference between SLE and RA patients?

Answer 21

RA will not be able to make a fist, SLE will

Question 22

What is suggestive of renal involvement in SLE?

Answer 22

Proteinurea > 500mg in 24 hours, red cell casts

Question 23

What test should always be performed when suspicious of SLE?

Answer 23

Urinalysis, if + then renal biopsy

Question 24

What feature of SLE gives an increased bleeding risk?

Answer 24

Thrombocytopenia

Question 25

People with SLE are very susceptible to infection. True or false?

Answer 25

True

Question 26

What three groups of auto-antibody are associated with SLE?

Answer 26

- Anti-Nuclear Antibody - Anti- Double Stranded DNA Antibody - Anti-Extractable Nuclear Antigens Antibody

Question 27

What are 4 anti-ENA antibodies which may be associated with SLE?

Answer 27

Anti-Ro, Anti-La, Anti-Sm, Anti-RNP

Question 28

Which two antibodies found in SLE are usually associated with cutaneous manifestations and secondary Sjogren's features?

Answer 28

Anti Ro and Anti La

Question 29

If Anti-Ro and Anti-La antibodies are present in a pregnant mother, what can this cause?

Answer 29

Premature lupus and foetal heart block

Question 30

Which Anti-ENA antibody is most specific for SLE?

Answer 30

Anti-Sm

Question 31

What is classed as a positive ANA test?

Answer 31

Positive in titre of 1:160 or higher

Question 32

Is ANA sensitive/specific/both for SLE?

Answer 32

Sensitive but not specific

Question 33

When should a positive ANA test be taken seriously?

Answer 33

When there are other autoantibodies present or when the patient presents with signs of CTD

Question 34

Is anti-double stranded DNA antibody specific for SLE?

Answer 34

Yes

Question 35

Concentrations of anti-double stranded DNA antibody vary with disease activity of SLE. True or false?

Answer 35

True

Question 36

What happens to complement levels during a flare of SLE?

Answer 36

They fall

Question 37

What drug will all patients with SLE be started on?

Answer 37

Hydroxychloroquine

Question 38

What are the main groups of drugs given for SLE?

Answer 38

NSAIDs and simple analgesia

Question 39

What dose of steroids should be given for an SLE patient with skin rashes, arthritis and serositis?

Answer 39

< 15mg/day prednisolone

Question 40

What dose of steroids should be given for an SLE patient with haematological involvement?

Answer 40

0.5mg/kg/day prednisolone

Question 41

What dose of steroids should be given for an SLE patient with resistant changes or major organ involvement?

Answer 41

1mg/kg/day prednisolone

Question 42

What is the most used immunosuppressant in SLE?

Answer 42

Cyclophosphamide

Question 43

What two biologics are options for SLE?

Answer 43

Rituximab, belimumab

Question 44

What is the treatment plan for mild SLE?

Answer 44

- Hydroxychloroquine - Topical Steroids - NSAIDs

Question 45

What is the treatment plan for moderate SLE?

Answer 45

- Hydroxychloroquine - Oral steroids - Azathioprine/methotrexate

Question 46

What is the treatment plan for severe SLE?

Answer 46

- Hydroxychloroquine - IV Steroids - Cyclophosphamide - Rituximab

Question 47

What are the two main features of APS?

Answer 47

- Venous or arterial thrombosis | - Adverse pregnancy outcomes

Question 48

What is the rash of APS known as?

Answer 48

Livedo reticularis

Question 49

What other conditions is APS associated with?

Answer 49

Any other autoimmune condition but especially SLE

Question 50

What are the 3 anti-phospholipid antibodies?

Answer 50

- Anti-Cardiolipin (IgM/IgG) - Lupus anticoagulant - Anti-Beta 2 Glycoprotein

Question 51

What is the rule with regards to autoantibodies to make a diagnosis of APS?

Answer 51

Must be positive on 2 occasions 12 weeks apart

Question 52

Do you have to be sensitive to all 3 antibodies for a diagnosis of APS?

Answer 52

No

Question 53

What lifelong therapy should APS patients be on?

Answer 53

Anticoagulation (aspirin/heparin) and hydroxychloroquine

Question 54

The diminished function of what causes the typical dry eyes and mouth of Sjogren's syndrome?

Answer 54

Lacrimal and salivary glands

Question 55

Who is Sjogren's syndrome most common in?

Answer 55

Women around 50s-60s

Question 56

Apart from the dry mouth/eyes etc, what are other clinical features of Sjogren's?

Answer 56

Joint Pains Fatigue Dental caries

Question 57

Which antibodies are most associated with Sjogren's?

Answer 57

Anti-Ro and anti-La

Question 58

Apart from the antibodies, what other tests can be done for Sjogren's?

Answer 58

Salivary gland ultrasound | Labial gland biopsy

Question 59

What is the general treatment for Sjogren's syndrome?

Answer 59

Artificial tears and saliva | Hydroxychloroquine

Question 60

Should immunosuppressants ever be given in Sjogren's?

Answer 60

Yes if there is major organ involvement

Question 61

What is the difference between diffuse and limited systemic sclerosis?

Answer 61

Limited- only really involves the skin | Diffuse- involves skin and other organ systems

Question 62

What antibodies are associated with diffuse systemic sclerosis?

Answer 62

- Anti-Topoisomerase - Anti-SCL-70 - Anti-RNA- III polymerase

Question 63

Where is the skin involvement in diffuse systemic sclerosis?

Answer 63

Proximal to forearms and torso

Question 64

Where is the skin involvement in limited systemic sclerosis?

Answer 64

Distal to elbows

Question 65

What are the CREST signs of limited systemic sclerosis?

Answer 65

- Calcinosis - Raynaud's - Oesophageal dysmotility - Sclerodactyly - Telangectasia

Question 66

What type of hypertension is related to systemic sclerosis?

Answer 66

Pulmonary

Question 67

What antibody is associated with limited systemic sclerosis?

Answer 67

Anti-centromere antibody

Question 68

What can you use to treat both Raynaud's and pulmonary hypertension?

Answer 68

Ca++ channel blockers

Question 69

What can you use to treat reflux?

Answer 69

PPIs, H2 antagonists

Question 70

What antibody is positive in mixed CTD?

Answer 70

Anti-RNP