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Year 3 > Systemic lupus erythematosus (MSK) > Flashcards

Systemic lupus erythematosus (MSK) Flashcards

1
Q

What is systemic lupus erythematosus (SLE)?

A

Multisystem autoimmune disease that predominantly affects women of childbearing age

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2
Q

What type of hypersensitivity reaction is SLE?

A

Type 3

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3
Q

Describe the epidemiology of SLE. (3)

A
  • F>M
  • onset usually at 20-40 years old
  • more common in Afro-Caribbeans
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4
Q

What causes SLE?

A

Genetic predisposition - HLA-DR2/3

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5
Q

Which genes predispose to SLE? (2)

A
  • HLA-DR2
  • HLA-DR3
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6
Q

What systems does SLE frequently involve?

A
  • most frequently involves skin and joints
  • serositis, nephritis, haematological cytopenias and neurological manifestations may occur during the course of the disease
  • pericarditis, myocarditis, pleuritis, pleural effusion
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7
Q

What is SLE characterised by?

A

Presence of antinuclear antibodies (ANA)

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8
Q

What syndrome is SLE associated with?

A

Anti-phospholipid syndrome - increased risk of thrombus formation (recurrent PEs and DVTs) and recurrent miscarriages

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9
Q

What are the clinical features of antiphospholipid syndrome (SLE)? (4)

A

CLOT

  • Clots (thrombosis)
  • Livedo reticularis
  • Obstetric complications (miscarriages)
  • Thrombocytopenia
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10
Q

What do investigations show in antiphospholipid syndrome (SLE)? (2)

A
  • prolonged APTT (paradoxical)
  • low platelets (thrombocytopenia)
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11
Q

What antibody is antiphospholipid syndrome (SLE) associated with?

A

Anti-cardiolipin antibodies

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12
Q

How is antiphospholipid syndrome (SLE) managed?

A
  • low dose aspirin (no previous thromboembolic event)
  • lifelong warfarin target INR 2-3 (after initial thromboembolic event) - avoid in pregnant women –> give aspirin and LMWH
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13
Q

What would adult-onset Still’s disease (SLE) present like (variant of juvenile rheumatoid arthritis)? (3)

A

Triad of:

  • joint pain
  • spiking fevers
  • pink bumpy rash
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14
Q

What are the clinical features of SLE?

A
  • malar (butterfly) rash - erythema over cheeks and bridge of nose, sparing nasolabial folds
  • photosensitive rash (after sun exposure - painful and pruritus for few days)
  • discoid rash - erythematous raised patches
  • fatigue, fever, weight loss
  • arthritis and arthralgia (distal symmetrical polyarthritis)
  • oral ulcers
  • pericarditis or myocarditis
  • Raynaud’s phenomenon
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15
Q

What is Raynaud’s phenomenon (SLE)?

A

Colour changes of the digits induced by cold: white –> blue –> red

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16
Q

How do we treat Raynaud’s phenomenon (SLE)?

A

Nifedipine

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17
Q

What rashes are seen in SLE? (3)

A
  • malar (butterfly) rash
  • photosensitive rash
  • discoid rash
18
Q

What are some extra-articular manifestations of SLE? (8)

A
  • pericarditis
  • myocarditis
  • pleuritis
  • pleural effusion
  • serositis
  • nephritis
  • haematological cytopenia
  • neurological manifestations
19
Q

What are some risk factors for SLE? (5)

A
  • female sex
  • age >30
  • African descent in Europe and US
  • drugs (e.g. procainamide, hydralazine)
  • family history of SLE
20
Q

What are the 1st-line investigations for SLE? (9)

A
  • ANA, anti-dsDNA, anti-Smith
  • ESR and CRP
  • FBC and differential
  • APTT
  • U&Es
  • urinalysis
  • CXR
  • ECG
  • C3/4 complement
21
Q

What is often used as a rule-out test for SLE?

A

Antinuclear antibody (ANA) - very sensitive (positive in most patients), but low specificity (may be positive in other conditions)

i.e. a positive ANA is not diagnostic

22
Q

When can ANA be negative in SLE?

A

Rarely, especially in anti-Ro-antibody-positive lupus

23
Q

What are some highly specific tests for SLE? (2)

A
  • anti-dsDNA
  • anti-Smith antibodies
  • (ANA sensitive but not specific)
24
Q

What is elevated in SLE according to disease activity?

A

ESR

25
Q

What is high in SLE if patient has an infection?

A

CRP (+ESR)

26
Q

What is decreased in SLE?

A

Complement levels C3/C4

27
Q

What do we see in drug-induced lupus (procainamide or hydralazine)?

A

Anti-histone antibodies

28
Q

Why do we do urinalysis in SLE?

A

Assess renal involvement - check for haematuria, proteinuria, casts (red cell, granular, tubular or mixed)

29
Q

What does FBC with differential show in SLE?

A
  • anaemia
  • leukopenia
  • thrombocytopenia
  • rarely pancytopenia
30
Q

What is APTT like in SLE?

A

May be prolonged in patients with antiphospholipid antibodies

31
Q

What are some differential diagnoses for SLE? (5)

A
  • rheumatoid arthritis
  • antiphospholipid syndrome
  • systemic sclerosis
  • mixed connective tissue disease
  • adult Still’s disease (variant of juvenile RA)
32
Q

What is the first-line management for SLE?

A

Hydroxychloroquine (also safe in pregnancy)

33
Q

What must be done before prescribing hydroxychloroquine (DMARD) in SLE?

A
  • visual acuity testing as can lead to severe and permanent retinopathy:
    • reduced colour differentiation
    • reduced central visual acuity
    • floaters
    • Bull’s eye maculopathy (red spot on macula surrounded by ring of retinal epithelial pigment loss)
  • baseline ophthalmological examination and annual screening generally recommended
34
Q

What is the general management for SLE? (4)

A
  • hydroxychloroquine (DMARD)
  • NSAIDs (naproxen)
  • avoid smoking
  • UV light
35
Q

What is used in SLE when hydroxychloroquine and NSAIDs are inadequate?

A

IV methylprednisolone

36
Q

What do we consider in SLE if internal organ involvement e.g. renal, neuro, eye?

A

Prednisolone, cyclophosphamide

37
Q

How do we manage severe SLE?

A
  • if organ threatening or refractory to hydroxychloroquine +/- corticosteroid: methotrexate or azathioprine or mycophenolate mofetil
  • consider belimumab or rituximab
38
Q

What is prescribed for skin manifestations in SLE?

A

Topical steroids (1% hydrocortisone)

39
Q

What are some complications of SLE? (7)

A
  • anaemia
  • leukopenia
  • thrombocytopenia
  • pericarditis
  • myocarditis
  • pleuritis
  • pleural effusion
40
Q

What are the most common causes of mortality in SLE? (3)

A
  • cardiovascular disease
  • followed by infection and severe disease activity

Year 3 (195 decks)

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