Systemic Lupus Erythematosus Flashcards

1
Q

What type of disease is systemic Lupus Erythematosus

A

Chronic autoimmune

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2
Q

Describe the epidemiology of systemic Lupus erythematosus

A

M:F = 1:9
15-40
Afro-Caribbean, Asian, Chinese

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3
Q

What does systemic Lupus erythematosus

A

Principally the joints and skin

Lungs, kidneys, haematology

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4
Q

Which genes are associated with systemic Lupus erythematosus

A
Multiple genes implicated
Complement deficiency e.g. C1q and C3 
Fc  receptors, IRF5, CTLA4, MHC class II HLA genes
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5
Q

Describe the presentation of systemic Lupus Erythematosus

A
Malaise
Fatigue 
Fever
Weight loss 
Lymphadenopathy
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6
Q

What are the specific features of systemic Lupus erythematosus

A

Butterfly rash
Alopecia
Arthralgia Raynaud’s phenomenon

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7
Q

What are some features of systemic Lupus erythematosus

A

Inflammation od kidney, CNS, heart, lungs
Accelerated atherosclerosis
vasculitis

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8
Q

Describe the pathogenesis of systemic Lupus erythematosus

A
  1. Genetic predisposition
  2. Environmental triggers
  3. Innate immune system activation
  4. Adaptive immune system activation
  5. Immune complexes
  6. (clinical disease onset)
  7. Aberrant amplification pathway
  8. Irreversible tissue damage
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9
Q

What are the laboratory tests done for Systemic Lupus Erythematosus

A
Antinuclear antibodies 
Anti-dsDNA and Sm
Anti-Ro and/or La
Haemotology 
Renal function
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10
Q

Describe the use of antinuclear antibodies in systemic Lupus Erythematosus

A

Relatively non-specific

Pattern is important

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11
Q

Describe the haematology of Systemic Lupus Erythematosus

A
Lymphopaenia
Normochromic anaemia
Leukopaenia
AIHA
Thrombocytopaenia
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12
Q

what are the renal function tests of Systemic Lupus Erythematosus

A

Proteinuria
Haematuria
Active urinary sediment

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13
Q

how is the disease severity of Systemic Lupus Erythematosus assessed

A

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

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14
Q

What are the clinical features that give signs of Systemic Lupus Erythematosus activity

A
Weight loss
Fatigue 
Malaise
Alopecia
Rash
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15
Q

What are the lab markers that give signs of Systemic Lupus Erythematosus

A

ESR
Increased complement consumption
Increase anti-dsDNA
Other Antibodies

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16
Q

Describe mild Systemic Lupus Erythematosus

A

Joint +/- skin involvement

17
Q

Describe moderate Systemic Lupus Erythematosus

A

Inflammation of other organs
Pleuritis
Pericarditis
Mild nephritis

18
Q

Describe severe Systemic Lupus Erythematosus

A
Severe inflammation in vital organs 
Severe nephritis
CNS disease
Pulmonary disease
Cardiac involvement 
AIHA, thrombocytopenia, TTP
19
Q

What is the treatment for mild Systemic Lupus Erythematosus and what is monitored

A

Paracetamol +/- NSAID (Monitor renal function)
Hydroxychloroquine
Topical corticosteroids

20
Q

What is the treatment for moderate Systemic Lupus Erythematosus

A

High dose corticosteroids

IV methylprednisolone

21
Q

What is the treatment for severe Systemic Lupus Erythematosus

A

Azathioprine

Cyclophosphamide

22
Q

What is the prognosis of Systemic Lupus Erythematosus

A

15 years:
no nephritis = 85%
nephritis = 60%
Prognosis worse if black, male + low socioeconomic status

23
Q

Describe the bimodal mortality pattern of Systemic Lupus Erythematosus

A

Early
Active lupus (renal failure, CNS disease)
Infection

Late
Myocardial infarction

24
Q

Describe the pathogenesis/pathophysiology of autoantibody formation in SLE

A
  1. Abnormal clearance of apoptotic cell material
  2. Dendritic cell uptake of auto antigens and activation of B cells
  3. B cell Ig class switching and affinity maturation
  4. IgG autoantibodies
  5. Immune complexes
  6. Complement activation, cytokine generation e.t.c