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MSK + Rheumatology > SLE - Systemic Lupus Erythematosus > Flashcards

SLE - Systemic Lupus Erythematosus Flashcards

1
Q

What is SLE?

A

Systemic Lupus Erythematosus.
An inflammatory multi-system disease characterised by the presence of serum anti-nuclear antibodies (ANA).

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2
Q

What kind of hypersensitivity reaction is lupus?
How is it mediated?

A

Type 3 hypersensitivity.
T, B, cells, cytokines and complement

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3
Q

Describe the epidemiology of SLE.

A
  • 90% of cases are in young women.
  • More common in afro-Caribbeans.
  • Peak age of onset between 20-40 yrs - typically women of childbearing age.
  • Genetic association.
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4
Q

Describe the pathophysiology of SLE.

A
  1. Inefficient phagocytosis -> means cell fragments + apoptotic cells aren’t cleared efficiently
  2. They are transferred to lymphoid tissue (LNs) where they are taken up by APCs.
  3. APCs present self-antigens from these fragments to T cells.
  4. T cells stimulate B cells, which proliferate to produce antibodies against self-antigens (auto-antibodies).
  5. Antibody formation -> Immune complex deposition -> complement activation -> neutrophil and cytokine influx -> inflammation and tissue damage
    = clinical manifestations.
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5
Q

What genetic associations is there in SLE?

A

HLA DR2
HLA DR 3
Complement C4 allele

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6
Q

Give 5 symptoms of SLE.

A
  1. Butterfly rash.
  2. Photosensitive rash
  3. Mouth ulcers.
  4. Raynaud’s phenomenon/’cold pale fingers’.
  5. Fatigue.
  6. Depression.
  7. Weight loss.
  8. Seizure.
  9. Psyhosis.
  10. Symptoms similar to RA for joints - symmetrical small joint
    arthralgia.
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7
Q

Give 5 signs of SLE.

A
  1. Glomerulonephritis - with persistent proteinuria
  2. Thrombocytopenia.
  3. Photosensitivity.
  4. Vasculitis.
  5. Anaemia.
  6. Deforming arthritis.
  7. Pericarditis.
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8
Q

What can cause thrombosis in patients with SLE?

A

The presence of antiphospholipid antibodies.

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9
Q

What investigations might you do in someone who you suspect has SLE?

A
  1. Blood count
    - May show neutropenia; thrombocytopaenia.
    - Anaemia of chronic disease or autoimmune haemolytic anaemia
    - Raised ESR, normal CRP.
    - Urea and creatinine ONLY raised if renal disease is advanced
  2. Serum autoantibodies:
    - ANA
    - Anti-dsDNA
  3. Serum Complement:
    - C3 and C4 reduced
  4. Histology
    - To see deposition of IgG and complement
  5. MRI and CT for lesions in brain
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10
Q

Describe the ESR and CRP levels in someone with lupus.

A

ESR is raised and CRP is low/normal.

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11
Q

What autoantibody is specific to SLE?

A

Anti-dsDNA.

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12
Q

What autoantibodies might you find in a patient with SLE?

A
  1. ANA - anti nuclear antibody.
  2. Anti-dsDNA - anti double-stranded DNA antibody.
  3. Anti-Ro, Anti-Sm and Anti-a.
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13
Q

What ESR/CRP results would make you think of SLE?

A

Multisystem disorder with raised ESR but normal CRP.

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14
Q

What histological features might you see on investigation of a patient with SLE?

A

Deposition of IgG and complement in kidneys and skin.

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15
Q

What are the WHO diagnostic criteria for SLE?

A

Need 4 out of 11 - SOAP BRAIN MD

Serositis
Oral ulcers
ANA +ve
Photosensitivity

Blood disorders
Renal disorder
Arthritis
Immunological disorder
Neurological disorder

Malar rash
Discoid rash

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16
Q

Describe the non-medical treatment for SLE.

A
  • Education and support.
  • UV protection.
  • Screening for organ involvement.
  • Reduce CVD risk factors e.g. smoking cessation and weight loss.
17
Q

Describe the pharmacological treatment for SLE.

A
  • Corticosteroids.
  • NSAIDS - for arthralgia, fever + malaise
  • Anti-malarials (DMARDs) - if NSAIDs don’t work.
  • Anticoagulants (for those with antiphospholipid antibodies).
  • Biological therapy targeting B cells e.g. rituximab.
18
Q

Outline the treatment of lupus. (5)

A

Manage triggers e.g. sunlight
NSAIDs for short term
Steroids for short term
Anti-malarial - Hydroxychloroquine
DMARDs - Methotrexate
TNF-a blocker - Rituximab

Warfarin - because antiphospholipid syndrome = greater risk of clotting

19
Q

How would you treat acute SLE e.g. haemolytic anaemia, nephritis, severe pericarditis or CNS disease?

A

Acute SLE e.g. haemolytic anaemia, nephritis, severe pericarditis or CNS disease:
* IV CYCLOPHOSPHAMIDE + HIGH DOSE PREDNISOLONE

20
Q

How would you manage the cutaneous symptoms of SLE?

A

For rashes:
Topical steroids, strong sunblock/avoid sun exposure

21
Q

How would you manage a severe flare of SLE?

A

IV cyclophosphamide and high-dose prednisolone.

22
Q

Describe maintenance treatment for SLE

A

NSAIDs, hydroxychloroquine, low-dose steroids, azathioprine/methotrexate or mycophenolate

23
Q

How would lupus nephritis be managed?

A

Needs more intense immunosuppression than SLE:
cyclophosphamide/mycophenolate

MSK + Rheumatology (22 decks)

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