Systemic Lupus Erythematosus Flashcards

1
Q

What population is SLE most common in?

A

15-40yo women

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2
Q

SLE principally affects… (2) but can also affect… (3)

A

joints and skin

lungs, kidneys, haematology

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3
Q

Presentation of SLE? (5)

A

Malaise, fatigue, fever, weight loss, lymphadenopathy

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4
Q

Specific features of SLE? (4)

A

Butterfly rash, alopecia, arthralgia, Raynaud’s phenomenon

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5
Q

Other features of SLE? (5)

A

Inflammation of kidney, CNS, heart and lungs, accelerated atherosclerosis, vasculitis

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6
Q

Whats used to diagnose SLE?

A

ACR criteria, 4/11 confirms diagnosis

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7
Q

A fundamental part of the pathogenic process in SLE is….

A

a deficiency of the clearance of apoptotic cells, they generate more apoptotic cells e.g. in response to UV light, anti-nuclear antibodies generated

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8
Q

pathogenesis of SLE? (6 steps)

A

abnormal clearance of apoptotic cell material -> dendritic cell uptake of auto antigens and activation of B cells -> B cell Ig class switching and affinity mutation -> IgG autoantibodies -> immune Complexes -> complement activation, cytokine activation etc

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9
Q

Lab tests for SLE? (3)

A

Antinuclear antibodies
Anti-dsDNA and Sm (antibodies against double stranded DNA will be positive in SLE, and anti-Sm will be seen in 20% of patients)
Anti-Ro and/or La (Ro is more important- these antibodies can cross the placenta and cause heart block in a fetus):

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10
Q

What does homogenous staining of ANAbs suggest?

A
  • Homogenous - Abs to DNA
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11
Q

What does speckled staining of ANAbs suggest?

A
  • Speckled - Abs to Ro, La, Sm, RNP
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12
Q

What does nucleolar staining of ANAbs suggest?

A
  • Nucleolar - topoisomerase – scleroderma
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13
Q

What does centromere staining of ANAbs suggest?

A
  • Centromere - limited cutaneous scleroderma
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14
Q

What haematological signs suggests SLE? (6)

A
  • Lymphopaenia, normochromic anaemia

- Leukopaenia, autoimmune haemolytic anaemia, thrombocytopaenia

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15
Q

What renal tests suggests SLE? (6)

A
  • Proteinuria, haematuria

- Active urinary sediment

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16
Q

3 criteria for assessing SLE severity?

A
  1. Identify pattern of organ involvement
  2. Monitor function of affected organs
     Renal - BP, U & E, urine sediment + Prot:Crea ratio
    Lungs/CVS - lung function, echocardiography
     Skin, haematology, eyes
  3. Identify pattern of autoantibodies expressed
    Anti-dsDNA, anti-Sm - renal disease
     Anti-cardiolipin antibodies
17
Q

SIGNS OF DISEASE ACTIVITY in SLE? (clinical features 6, lab markers 4)

A
  • Weight loss, fatigue, malaise, hair loss
  • Alopecia
  • Rash
  • ESR ESR will go up with active disease but CRP will stay flat (both are raised in rheumatoid arthritis)
  • Increased complement consumption
  • Increased anti-dsDNA
  • Other Abs e.g. ANA (not a marker of disease activity) and CRP poor indicators
18
Q

ESR in SLE?

A

Increased

19
Q

CRP in SLE?

A

Flat

20
Q

Complement levels in serum in SLE?

A

Diminished

21
Q

Serum anti-dsDNAab in SLE?

A

Increased

22
Q

Treatment of mild disease SLE? (3)

A

A. Paracetamol +/- NSAID
B. Hydroxychloroquine Can cause retinal problems
C. Topical corticosteroids

23
Q

What is hydroxychloroquine used for and what side effect must you be aware of?

A

SLE and retinal problems

24
Q

Treatment of moderate disease SLE?

A

Corticosteroids

  • high initial dose to suppress disease activity (0.5-1.5mg/kg/day)
  • i.v. methylprednisolone 3 x 0.5-1g per 24h
  • initial oral dose for 4 weeks
  • reduce slowly over 2-3 mths to 10 mg/d
  • reduce slowly at 1mg per month
25
Q

What indicates moderate SLE?

A
  • failure of hydroxychloroquine/NSAID

- organ/life threatening disease

26
Q

Treatment of severe disease SLE? (4)

A

Azathioprine
Cyclophosphamide
Mycophenolate mofetil
Rituximab and belimumab

27
Q

Effect of Mycophenolate mofetil? Benefit of it?

A
  • Reversible inhibitor of inosine monophosphate dehydrogenase
  • Rate-limiting enzyme in de novo purine synthesis
  • Lymphocytes – dependent upon de novo purine synthesis
  • Won’t impair fertility
28
Q

Effect of rituximib?

A
  • Anti-CD20 depletes B cell
29
Q

Early mortality causes of SLE? (3)

A

Renal failure, CNS disease, infection

30
Q

Late mortality cause of SLE?

A

MI

31
Q

What defines mild SLE?

A

joint +/- skin involvement

32
Q

What defines moderate SLE? (3)

A

inflammation of other organs- pleuritis, pericarditis, mild nephritis

33
Q

What defines severe SLE? (5)

A

severe inflammation in vital organs:

  • severe nephritis
  • CNS disease
  • pulmonary disease
  • cardiac involvement
  • AIHA, Thrombocytopaenia, TTP