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ortho & rheum > SYSTEMIC LUPUS ERYTHMATOSUS > Flashcards

SYSTEMIC LUPUS ERYTHMATOSUS Flashcards

1
Q

Define systemic lupus erythmatosus.

A

Systemic lupus erythematosus (SLE) is a heterogeneous, inflammatory, multisystem autoimmune disease in which antinuclear antibodies occur (often years before clinical symptoms).

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2
Q

What are the defects in SLE?

A

Failure of self tolerance by the T and B cells

Defects in apoptosis system

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3
Q

Which virus has been implicated as a possible trigger for SLE?

A

EBV

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4
Q

To make a positive diagnosis of SLE what are the antibodies that we are looking?

A

Anti-nuclear antibodies (ANA)

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5
Q

Are ANA’s specific to SLE?

A

No. The different subtypes are present in a number of connective tissue disorders including Sjögren’s syndrome, scleroderma, mixed connective tissue disease, polymyositis, dermatomyositis, autoimmune hepatitis and drug induced lupus.

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6
Q

What is c1q?

A

It is a subtype of compliment involved in clearing debris of apoptosic cells.

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7
Q

How might a patient with suspected SLE present? (Name 3 symptoms)

A

Butterfly rash
Fatigue
Joint pain

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8
Q

Are CRP and ESR both classically raised in SLE?

A

No. Normally only ESR is raised. However, if there is infection at the time, then CRP may well be raised.

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9
Q

What are some of the monoclonal antibodies that are used in the treatment of SLE?

A

Rituximab
Belimumab
Erpratuzamab - currently undergoing trials

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10
Q

What is the peak age for females to develop symptoms of SLE?

A

50-54

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11
Q

What is the peak age for males to develop symptoms of SLE?

A

70-74

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12
Q

What is the ratio of women to men in terms of those affected by SLE?

A

10:1 - 5:1

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13
Q

What are the risk factors for developing SLE?

A 
Certain HLA-DRB1 types e.g DR3 and DR2
Also HLA-B8
Defective C4 complement gene - develop a lupus-like illness
UV light
EBV
Drugs
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14
Q

What are the drugs that can induce Lupus?

A

Chlorpromazine - antipsychotic
Methyldopa - Parkinson’s
Hydralazine - Anti-hypertensive
Isoniazid - TB
d-Penicillamine - Immunosuppressive for RA
Minocycline - broad spectrum tetracycline antibiotic

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15
Q

How might someone with suspected SLE present to their GP?

A 
Fatigue
Malaise
Fever
Weight loss
Arthralgia with early morning stiffness
Oral ulcers
Photosensitive skin rashes - malar rash, urticaria
Pleuritic chest pain
Headache
Paraesthesia
Dry eyes
Dry Mouth
Raynaud's phenomenon
Mild hair loss
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16
Q

What part of the face does the classic malar rash seen in SLE not affect?

A

Naso-labial fold

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17
Q

What is Raynaud’s phenomenon?

A

Excessively reduced blood flow in response to cold or stress, causing discoloration (white then blue then red) of fingers.

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18
Q

Which connective tissue disorders are associated with Raynaud’s phenomenon?

A 
Scleroderma
Systemic lupus erythematosus
Rheumatoid arthritis
Sjögren's syndrome
Dermatomyositis
Polymyositis
Mixed connective tissue disease
Cold agglutinin disease
Ehlers-Danlos syndrome
19
Q

What is the possible underlying pathophysiology of SLE?

A

Apoptotic cells and cell fragments inefficiently cleared by phagocytes.
They are then transferred to lymphoid tissue- taken up by APCs.

Self antigens including nuclear constituents eg: DNA and histones- these are presented to T cells- they stimulate B cells to produce auto-antibodies.

Clinical manifestations due to antibody formation and development and deposition of immune complexes, complement activation and influx of neutrophils and abnormal cytokine production.

20
Q

Specifically which cytokines are increased in serum concentration in SLE?

A

IL-10

IFN-α

21
Q

Which population (in terms of race) is most at risk of developing SLE?

A

Afro-Caribbean

22
Q

What is discoid lupus?

A

A benign variant of SLE where only the skin is involved. So facial rash with erythematous plaques.

23
Q

What are the 11 parts of the diagnostic criteria of SLE and how many does the criteria require the patient to have?

A

1) Malar flush
2) Discoid rash
3) Photosensitivity
4) Oral ulcers
5) Non erosive arthritis
6) Serositis
7) Renal disorder
8) CNS disorder
9) Haematological disorder
10) Immunological disorder
11) Antinuclear antibody (+ve in >95%)

4 or more of the 11

24
Q

On examination what signs might you pick up in someone with suspected SLE?

A 
Very non specific
Splenomegaly
Lymphadenopathy
Rashes
Any signs associated with complications of SLE
25
Q

What investigations would you order for someone with suspected SLE?

A 
Blood test:
FBC
ESR
CRP
Autoantibodies

Other investigations depending on involvement from specific body parts.

26
Q

What might the FBC reveal in someone with suspected SLE?

A

Normochromic, normocytic anaemia.
Leukopenia
Thrombocytopenia

27
Q

What pattern of results with regard to ESR and CRP would you expect to see in someone with SLE?

A

ESR raised but normal CRP unless there is intercurrent infection or serositis.

28
Q

What is the most sensitive autoantibody present in patients with SLE, with 95% of all patients having this auto-antibody?

A

ANA

29
Q

Which auto-antibody can be used to reflect disease activity in the 70% of SLE patients that have this auto-antibody?

A

Anti-dsDNA - high specificity but only 70% sensitivity.

A rise in antibody titre might indicate that immunosuppression needs to be increased.

30
Q

What is the most specific antibody present in 30-40% of patients with SLE?

A

Anti-Sm

31
Q

Which antibody, also called anti-SSA, is associated with several autoimmune diseases such as SLE and Sjogren’s syndrome?

A

Anti-Ro

32
Q

Which antibody might indicate mixed connective tissue disease with overlap of SLE, scleroderma and myositis?

A

Anti-RNP

33
Q

Which anti-mitochondrial antibody is sometimes found in SLE patients, as well as syphillis and anti-phospholipid syndrome?

A

Anti-cardiolipin

34
Q

How are levels of complement affected in SLE patients and specifically which forms of complement are affected?

A

C3 and C4 are decreased

C3d is actually increased with disease activity

35
Q

What type of complement is C3d, often found to be increased with increase in disease activity in SLE patients?

A

A degradation product

36
Q

What are the complications associated with SLE?

A 
Atherosclerosis
Hypertension
Dyslipidaemias
Diabetes
Osteoporosis
Avascualar necrosis
Malignancy
37
Q

Which malignancy is especially associated with SLE?

A

Non-Hodgkin’s lymphoma

38
Q

What are the pharmacological treatment options for managing a SLE patient?

A

Paracetamol NSAIDs for joint and muscle pain.
Corticosteroids (some risks though)
Hydroxychloroquine (malaria tablets) - useful for skin lesions, myalgia and malaise

39
Q

What drugs might be given to someone with life-threatening SLE?

A 
Cyclophosphamide
Mycophenolate
High dose prednisolone
Methatrexate
Ciclosporin
Belimumab (B-cell inhibitor)
Intravenous immunoglobulins
40
Q

What non-pharmacological form of therapy can be offered to a SLE patient with life-threatening disease?

A

Plasma exchange

41
Q

What are the leading causes of death in SLE patients?

A 
Renal failure
Cerebral disease (often epilepsy)
Infection
Cardiovascular disease related to atherosclerosis
Malignancy (Non-Hodgkins Lymphoma)
42
Q

Which antibody is often used as an indicator of drug induced lupus?

A

Anti-histones

43
Q

Which antibody, also called Anti-SSB, is mainly associated with Sjogren’s syndrome but is also present is about 15% of SLE patients?

A

Anti-La

ortho & rheum (28 decks)

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