Connective tissue disease 1

Question 1

SLE is commercial in African Americans T/F

Answer 1

T

Question 2

Male to female ratio for SLE in 9-1 T/F

Answer 2

F- commercial in women. however if it does occur in men it tends to be more severe

Question 3

Examples of CTD

Answer 3

SLE
Sjogen's syndrome
Sytemic sclerosis
Dermatomyosititis
Polymyosititis
Mixed connective tissue disease
Anti-phospholipid syndrome

Question 4

Connective tissue diseases are diseases of the connective tissue T/F

Answer 4

F- characterised by the presence of over activity of the immune system

Question 5

What is SLE

Answer 5

Immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage

Question 6

Why does kidney disease cause so many deaths?

Answer 6

It often does not cause any symptoms so can go unnoticed ( even though very severe- kidney can stop working entirely)

Question 7

Which three factors are inportant in the aetiology of SLE and why?

Answer 7

Genetic- monozygotic twins, relatives, gene abnormalities
Hormonal- higher oestrogen exposure(early menarche) , on oestrogen containing contraceptives and HRT
Environmental- Viruses, UV light( may stimulate skin to release cytokines stimulating B cells) , Silica dust

Question 8

What is the pathogenesis of SLE

Answer 8

Increased and defective apoptosis
necrotic cells release nuclear material which acts as potent auto-antigens
Autoantibody probably as a result of extended exposure to nuclear and intracellular organisms
B cells and T cells stimulated
Auto-antibodies produced

Question 9

What are constitutional symptoms?

Answer 9

Very common - but not diagnostic ( as they are so common)

Question 10

Why is there renal disease with SLE?

Answer 10

deposition of immune complexes in mesangium
Comlexes consist of nuclear antigens and anti-nuclear antibodies
Complexes form in circulation then are depoisited
Once present they active competent which activtes leukocytes which releases cytokines.
Cytokines release perpetuates inflammation which, over time, causes necrosis and scarring

Question 11

Describe the rash associated with SLE

Answer 11

spares the nasallabial folds
Lasts long time
butterfly

Question 12

How is Jaccoud’s arthritis different to —-. SECTIONMISSING

Answer 12

Reversible and if x-rays there is no change to the joints

Question 13

What are the mucocutaneous features of SLE?

Answer 13

Photosensitivity
Malar rash
Discoid lupus erythematous( may scar)
Subacute cutaneous lupus
Mouth ulcers ( painless)
Alopecia ( non-scarring)

Question 14

What are you looking for in terms of SLE in a dipstick

Answer 14

Looking for blood or protein in urine

Question 15

What are the MSK features of SLE

Answer 15

Non-deforming polyarthiritis/polyarthralgia (no radiological erosion)
Deforming arthropathy-Jaccoud’s arthritis
Erosive arthritis-rare
Myopathy-weakness,myalgia and myosititis

Question 16

What is serosititis and what problems can it cause?

Answer 16

inflammation of the serous tissues of the body, the tissues lining the lungs (pleura), heart (pericardium), and the inner lining of the abdomen (peritoneum) and organs within.

Pericarditis
Pleurisy
Pleural effusion
Pericardial effusion

Question 17

What are the neurological features of SLE?

Answer 17

Depression/psychosis
migraninous headache
seizures
cranial or peripheral neuropathy
mononeuritis multiplex

Question 18

What are the haematological features of SLE

Answer 18

lymphadenopathy -(25% of patients)
Leucopenia
Lymphopenia
Haemolytic anaemia
Thrombocytopenia

Question 19

Organ involvement that involves treatment but is not life threatening- where would this lie on the spectrum of disease

Answer 19

Moderate

mild- may not need treatment, severe os life threatening

Question 20

What is anti-phospholipid syndrome

Answer 20

Venous and arterial thrombosis
Recurrent miscarriage
Lividio recticularis
Assocaitions with autoimmune conditions especially SLE
Thrombocytoenia
Prolonged APTT

Question 21

Why doe so many people with CTD have infections?

Answer 21

Intrinsic-low complement, impaired cell mediated immunity, defective phagocytosis, poor antibody response to certain antigens

Exrtinsic-Steroids, other immunosuppression drugs, nephrotic syndrome

Question 22

What is ANA

Answer 22

Anti-nuclear antibody
High titre In 95% of SLE patients
Low titre in 20% of population
Found in rheumatoid arthritis and other autoimmune conditions, HIV, hep c

Question 23

When should a positive NA be taken seriously?

Answer 23

if other antinuclear antibodies are positive
Anti-dsDNA
Anti-Sm
Anti-Ro
Anti-RNP

When the patient presents with CTD features

Question 24

What is anti-double stranded DNA antibody?

Answer 24

Occurs in 60% of patients with SLE-Highly specific
Titre correlated with overall disease activity
may be associated with lupus nephritis

Question 25

What antibody is most specific to SLE

Answer 25

Anti-double stranded DNA antibody

Question 26

What is Anti-ENA

Answer 26

Anti-Ro (60%) Usually associated with anti-La Associated with cutaneous manifestations Secondary Sjogen's features Congenital heart block and neonatal LE Anti-Sm highly specific (10-20%)- probable neurological Anti-RNP(30%)- overlap potentially with sclerodermatous skin lesions, Raynaud's pnenonenom, low grade myosis

Question 27

What would be the most appropriate first investigation in a patient with suspect SLE

Answer 27

Urinalysis

Question 28

What are Anti-phospholiod antibodies

Answer 28

Anti-cardiolipin antibody Lupus anticoagulant Anti-beta 2 glycoprotein Must be positive on 2 occasions 12 weeks apart

Question 29

Normally as disease flares CRP increases. What disease is an exception to this?

Answer 29

SLE - it CRP is high indicated infection

Question 30

Once diagnosis is established...

Answer 30

Essential to screen for organ involvement

Question 31

What other investigations may be necessary?

Answer 31

``` CXR pulmonary function tests CT chest Urine protein quantification Renal biopsy Echocardiogram Nerve conduction studies MRI brain ```

Question 32

What is the drug that everyone with SLE gets

Answer 32

Hydroxychloroquine- useful for arthritis,cutaneous manifestations and constitutional symptoms. may reduce sys complications

Question 33

How do you monitor levels of SLE activity?

Answer 33

``` Clinical assessment inc BP Anti-dsDNA level pos correlates C3/C4 level neg correlates Urien exam inc portens, cells and casts Full blood count Blood biochemistry ```

Question 34

What is the general management of SLE

Answer 34

Councelling- patients, spouse and relatives Regular monitoring Avoid excessive sun-exposure Pregnancy issues

Question 35

When are steroids used?

Answer 35

``` Steroids could be used for inflammatory drug problems etc. Small doses for rates,arthiritis and serotitis Moderate for resistant serosititis, haematological abnormalities and class V GN High doses for severe/resistant haematological changes, diffuse GN and major organ involvement ```

Question 36

What is the most common immunosuppressive used?

Answer 36

-Cyclophosphamide (methotrexate can also be used)

Question 37

What do you use to treat a mild disease?

Answer 37

HCQ Topical steroids NSAIDs

Question 38

Moderate disease treatment

Answer 38

Oral steroids Azathioprine Methotexate

Question 39

Severe disease treatment

Answer 39

IV steroids Cyclophosphamide Rituximab

Question 40

What are the biologics that can be used in SLE

Answer 40

Anti-CD20 (Rituximab) | Anti-Blys (Belimumab)