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LCRS Musculoskeletal > Systemic lupus erythematosus > Flashcards

Systemic lupus erythematosus Flashcards

1
Q

Lupus is in a family of overlapping autoimmune diseases, what other autoimmune diseases are in the family?

A 
Rheumatoid arthritis
Sjogren's syndrome
Dermatomyositis
Polymyositis
Systemic sclerosis
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2
Q

What is the distribution of lupus like in terms of gender and age?

A

M:F 1:9 Presentation 15-40 years

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3
Q

In what races is there increased prevalence of lupus

A

Afro-caribbean, asian and chinese

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4
Q

What does lupus principally effect?

A

Joints and skin
Lungs
Kidneys
Haematology

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5
Q

What genetic associations does lupus have?

A 
Multiple genes implicated
Complement deficiency e.g. C1q and C3
Fc receptors, IRF5, CTLA4, MHC class II HLA genes
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6
Q

How does lupus normally present?

A 
Malaise
Fatigue
Fever 
Weight loss
Lymphadenopathy
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7
Q

What specific features are there of lupus presentation?

A

Butterfly rash
Alopecia
Arthralgia
Long history of Raynaud’s phenomenon

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8
Q

What other features are there of lupus?

A

Inflammation of kidney, CNS, heart and lungs
Accelerated atherosclerosis
Vasculitis

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9
Q

Why is lupus a very difficult disease to diagnose?

A

It affects so many different systems

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10
Q

How do lupus patients appear?

A

The disease makes patients feel incredibly unwell
They have rashes- nose/chin, it may look a bit like acne- localised inflammation but isn’t itchy or painful.
Some rashes can become depigmented- this happens when inflammation extends to the dermis
Depigmentation and scarring is irreversible- however this is usually quite rare

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11
Q

What are the immune systems of SLE patients like normally?

A

They tend to be overactive- particularly humoral

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12
Q

How is apoptosis affected in lupus?

A

SLE patients have a defect in apoptosis
Normally we clear dead cells in a non-inflammatory way however patients with SLE can’t do this
There is impaired clearance that is associated with inflammation. Apoptotic cells linger in the body and expose nuclear antigens on their surface.
Auroantibodies

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13
Q

What happens to the nuclear antigens that are exposed on the surface?

A

Autoantibodies are generated against them

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14
Q

What is the defect in apoptosis combined with and what does this lead to?

A

B cell hyperactivity- overactive B cells are exposed to autoantigens and plasma cells begin to produce autoantibodies that circulate and form immune complexes.

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15
Q

Due to the B cell hyperactivity, what happens to the immune complexes formed?

A

They deposit in tissues (mainly kidneys and skin) and activate complement in the tissue

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16
Q

Give a brief summary of autoantibody production in lupus?

A

Abnormal clearance of apoptotic cell material
Dendritic cell uptake of autoantigens and activation of B cells
B cell Ig class switching and affinity mutation
IgG autoantibodies
Immune complexes
Complement activation and cytokine generation etc

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17
Q

What is the first thing you do to diagnose SLE?

A

First thing you do is send serum to check for anti-nuclear antibodies (ANA)- useful but not diagnostic

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18
Q

How does ANA testing work?

A

Serum is placed on a glass slide with cells suspended on it and if ANAs are present they will bind to nuclear antigens.
To tell whether they have bound, you add fluorescently labelled monoclonal antibody that binds to the ANA and observe pattern of attachment

19
Q

What are the different patterns of ANA and what do they mean?

A

Homogenous- Abs to DNA- SLE
Speckled- Abs to Ro, La, Sm and RNP- SLE overlap syndromes
Nucleolar- topoisomerase- scleroderma
Centromere- limited cutaneous scleroderma

20
Q

How does usefulness of Anti-dsDNA and Sm testing differ to ANA?

A

More specific but less sensitibe

21
Q

What is anti-Ro and/or La testing used for?

A

Common in subacute cutaneous LE

Neonatal lupus syndrome and Sjogren’s

22
Q

What other tests are used to diagnose lupus?

A

INcreased complement consumption
Anti-cardiolipin antibodies
Lupus anticoagulant
Beta1 glycoprotein

Haematology-
Lympopaenia, normochromic anaemia
Leukopaenia, AIHA, thrombocytopenia 
Renal- 
Proteinuria, haematuria
Active urinary sediment
23
Q

How does complement consumption change in lupus?

A

Patients with active lupus form immune complexes and they fix complement and consume it, so if you measure complement in the blood (c3 and c4) it will be low in active lupus

24
Q

What is happening in autoimmune haemolytic anaemia?

A

Antibodies are binding to red cell surface and are being taken out in the spleen

25
Q

How do you assess severity of lupus?

A

Identify pattern of organ involvement
Monitor function of affected organs
Identify pattern of autoantibodies expressed

26
Q

What do you monitor in terms of renal and lungs/CVS

A

Renal- BP, U and E, urine sediment and prot:crea ratio

Lungs/CVS- Lung function and ECG

27
Q

What else do you monitor function of?

A

Skin, haematology and eyes

28
Q

What pattern of autoantibodies preempts renal disease?

A

High titre anti-dsDNA and anti-Sm autoantibodies

29
Q

What signs of disease activity are there in terms of clinical and laboratory markers?

A 
Clinical-
Weight loss, fatigue and malaise
Alopecia
Rash
Laboratory-
ESR
Increased complement consumption
Increased anti-dsDNA
Other Abs e.g. ANA and CRP poor indicators
30
Q

What is the importance in picking up disease activity early?

A

You could prevent organ damage

31
Q

How is lupus classified into 3 groups?

A

Based on severity:
Mild- Joint and/or skin involvement
Moderate- Inflammation of other organs, pleuritis, pericarditis, mild nephritis
Severe- Severe inflammation in vital organs

32
Q

What does severe lupus consist of?

A 
Severe nephritis 
CNS disease
Pulmonary disease
Cardiac involvement
AIHA, thrombocytopenia and TTP
33
Q

How do you treat mild disease?

A

Paracetamol +/- NSAID- monitor renal function
Hydroxychloroquine- arthropathy, cutaneous manifestations and mild disease activity
Topical corticosteroids

34
Q

What are the indications of moderate disease?

A

Failure of hydroxychloroquine/NSAID

Organ/life threatening disease

35
Q

How do you treat moderate disease?

A 
Corticosteroids:
High initial dose to suppress disease activity
IV methylprednisolone 3x 0.5-1g per 24hr
Initial oral dose for 4 weeks
Reduce slowly over 2-3 months to 10mg/d
Reduce slowly to 1mg per month
36
Q

Why do you give steroid sparing agents as well as steroids when treating moderate disease?

A

High dose steroids are very toxic so may give steroids with steroid sparing agents so you can lower dose

37
Q

How do you treat severe disease?

A 
Azathioprine:
Moderate to severe disease 2.5mg/kg/day
Effective steroid sparing agent
20% neutropenia
Regular FBC and biochem monitoring

Cyclophosphamide-
Severe organ involvement, IV pulsed or oral Rx
BM suppression, infertility and cystitis

38
Q

What new treatment is there for severe disease?

A

Mycophenolate mofetil:
Reversible inhibitor of inosine monophosphate dehydrogenase which is a rate-limiting enzyme in de novo purine synthesis- lymphocytes are dependent on this

Rituximab:
Anti-CD20 mAb therapy that leads to depletion of B cells
It is effective in lupus nephritis

39
Q

What effect does mycophenolate mofetil and rituximab have on fertility?

A

None but it is teratogenic so shouldn’t try to conceive

40
Q

What is the prognosis and survival like in severe lupus?

A

15 year survival:
No nephritis- 85%
Nephritis- 60%
Worse if black, male or low socio-economic status

41
Q

What is the mortality pattern of lupus like?

A

There is the bimodal mortality pattern as there is an early peak in mortality and then a later peak as well

42
Q

What causes the early and late peaks in mortality?

A 
Early-
Renal failure
CNS disease
Infection
Late-
Myocardial infarction
Stroke
43
Q

What could the blood film of a patient with SLE show?

A 
Schistocytes (fragments)- microangiopathic haemolytic anaemia
Spherocytes
Tear drop cells
Few leukocytes
Few platelets
44
Q

What could the renal biopsy of a patient with SLE show?

A

Hypercellular- loads more cells than in a normal glomerulus
Mesangial proliferation
Crescent development- inflammatory cells that have migrated into glomerulus

LCRS Musculoskeletal (7 decks)

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