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MSK week 2 > Connective tissue disease 1 > Flashcards

Connective tissue disease 1 Flashcards

1
Q

SLE is commercial in African Americans T/F

A

T

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2
Q

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

A

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

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3
Q

Examples of CTD

A 
SLE
Sjogen's syndrome
Sytemic sclerosis
Dermatomyosititis
Polymyosititis
Mixed connective tissue disease
Anti-phospholipid syndrome
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4
Q

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

A

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

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5
Q

What is SLE

A

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

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6
Q

Why does kidney disease cause so many deaths?

A

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

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7
Q

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

A

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

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8
Q

What is the pathogenesis of SLE

A

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

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9
Q

What are constitutional symptoms?

A

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

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10
Q

Why is there renal disease with SLE?

A

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

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11
Q

Describe the rash associated with SLE

A

spares the nasallabial folds
Lasts long time
butterfly

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12
Q

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

A

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

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13
Q

What are the mucocutaneous features of SLE?

A 
Photosensitivity
Malar rash
Discoid lupus erythematous( may scar)
Subacute cutaneous lupus
Mouth ulcers ( painless)
Alopecia ( non-scarring)
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14
Q

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

A

Looking for blood or protein in urine

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15
Q

What are the MSK features of SLE

A

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

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16
Q

What is serosititis and what problems can it cause?

A

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

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17
Q

What are the neurological features of SLE?

A 
Depression/psychosis
migraninous headache
seizures
cranial or peripheral neuropathy
mononeuritis multiplex
18
Q

What are the haematological features of SLE

A 
lymphadenopathy -(25% of patients)
Leucopenia
Lymphopenia
Haemolytic anaemia
Thrombocytopenia
19
Q

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

A

Moderate

mild- may not need treatment, severe os life threatening

20
Q

What is anti-phospholipid syndrome

A 
Venous and arterial thrombosis
Recurrent miscarriage
Lividio recticularis
Assocaitions with autoimmune conditions especially SLE
Thrombocytoenia
Prolonged APTT
21
Q

Why doe so many people with CTD have infections?

A

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

Exrtinsic-Steroids, other immunosuppression drugs, nephrotic syndrome

22
Q

What is ANA

A

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

23
Q

When should a positive NA be taken seriously?

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

When the patient presents with CTD features

24
Q

What is anti-double stranded DNA antibody?

A

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

25
Q

What antibody is most specific to SLE

A

Anti-double stranded DNA antibody

26
Q

What is Anti-ENA

A

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

27
Q

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

A

Urinalysis

28
Q

What are Anti-phospholiod antibodies

A

Anti-cardiolipin antibody
Lupus anticoagulant
Anti-beta 2 glycoprotein

Must be positive on 2 occasions 12 weeks apart

29
Q

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

A

SLE - it CRP is high indicated infection

30
Q

Once diagnosis is established…

A

Essential to screen for organ involvement

31
Q

What other investigations may be necessary?

A 
CXR
pulmonary function tests
CT chest
Urine protein quantification
Renal biopsy
Echocardiogram
Nerve conduction studies
MRI brain
32
Q

What is the drug that everyone with SLE gets

A

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

33
Q

How do you monitor levels of SLE activity?

A 
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
34
Q

What is the general management of SLE

A

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

35
Q

When are steroids used?

A 
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
36
Q

What is the most common immunosuppressive used?

A

-Cyclophosphamide (methotrexate can also be used)

37
Q

What do you use to treat a mild disease?

A

HCQ
Topical steroids
NSAIDs

38
Q

Moderate disease treatment

A

Oral steroids
Azathioprine
Methotexate

39
Q

Severe disease treatment

A

IV steroids
Cyclophosphamide
Rituximab

40
Q

What are the biologics that can be used in SLE

A

Anti-CD20 (Rituximab)

Anti-Blys (Belimumab)

MSK week 2 (11 decks)

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