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Management of Disorders > Systemic Lupus Erythematosus > Flashcards

Systemic Lupus Erythematosus Flashcards

1
Q

What does SLE stand for?

A

Systemic Lupus Erythematosus

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2
Q

What is SLE?

A

A chronic autoimmune disease that causes the overreactivity of the immune system, producing auto antibodies, leading to systemic inflammation of multiple organs

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3
Q

What is the famous sign of lupus?

A

A butterfly rash on the face

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4
Q

Where are rashes localised to?

A

Sun exposed areas

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5
Q

How long do rashes last for?

A

Days or weeks

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6
Q

What demographic is most likely to be affected by lupus?

A

Non-Caucasian females, age 15-45

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7
Q

Why do likely see more cases of lupus amongst women ages 15-45

A

Sex hormones

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8
Q

Name two possible environmental triggers for lupus.

A

Hormones and UV light (sun exposure)

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9
Q

Does genetic risk of lupus put you at higher risk for other autoimmune disorders?

A

Yes

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10
Q

Does lupus have a genetic component?

A

Yes, although this is complex

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11
Q

How much does your risk for lupus increase if you have a first degree relative with the condition?

A

6x

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12
Q

What is the laboratory hallmark of lupus?

A

Antinuclear antibodies (ANA ) - present in 98% of patients

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13
Q

Which autoantibody shows a correlation with lupus disease activity?

A

dsDNA

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14
Q

Why is dsDNA not the first marker to look for in diagnosing lupus?

A

Because 30-50% of people with SLE don’t have it.

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15
Q

What might the presence of Sm autoantibodies suggest?

A

SLE with renal involvement

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16
Q

What is MCTD?

A

Mixed connective tissue disease - an autoimmune disease that shares features of lupus

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17
Q

What can RNP be used as a marker for?

A

MCTD and overlapping symptoms such as renal involvement, Raynauds and lung disease

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18
Q

What are Ro autoantibodies associated with?

A

Sjorgren’s syndrome (dry mouth, salivary issues), skin-related lupus, and neonatal lupus

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19
Q

What are antiphospholipid antibodies associated with?

A

Thrombosis complications, pregnancy loss, and neurological conditions such as myelitis

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20
Q

What is ribosomal P associated with?

A

Psychiatric disease

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21
Q

What does APS stand for?

A

Anti-phospholipid syndrome

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22
Q

What are the three main signs of APS?

A

Arterial and/or venous thrombosis, pregnancy morbidity, and antiphospholipid antibodies

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23
Q

What is the main treatment for vascular disease in SLE?

A

Immunosuppression

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24
Q

What is the main treatment for vascular disease in APS?

A

Anticoagulation

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25
Q

What condition can cause vascular disease that affects small vessels and is vasculitic in nature?

A

SLE

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26
Q

How does vascular disease in APS differ from that in SLE?

A

It is a coagulation disorder that affects both the large and small vessels, and is thrombotic in nature

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27
Q

How can SLE cause vascular disease?

A

Overreactivity of the immune system can cause lesions in the small vessels, damaging them (immune complex mediated)

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28
Q

Can someone have both SLE and APS?

A

Yes

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29
Q

What is APS?

A

An immune system disorder where antibodies are created that make much blood more likely to clot

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30
Q

What are some of the most common symptoms of SLE?

A

▪️Fatigue
▪️Arthritis/arthralgia
▪️Skin signs (e.g rashes)
▪️Fever
▪️Raynaud’s
▪️Swelling of limbs and joints

Can affect all organs!

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31
Q

Are antinuclear antibodies specific to SLE?

A

No!

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32
Q

What laboratory signs are looked for to diagnose definite APS?

A

▪️Lupus anticoagulant (LA)
▪️IgG/IgM aCL (medium/high titre)
▪️IgG/IgM anti-b2GPI

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33
Q

What SLE/APS related factors increase risk of stroke?

A

▪️SLE itself
▪️Antiphospholipid antibodies
▪️Vasculopathy/vasculitis
▪️Accelerated atherosclerosis through prolonged steroid use
▪️Valvular disease

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34
Q

How might an acute confusional state present in SLE?

A

▪️Fluctuating level of consciousness
▪️Acute onset
▪️Often related to infection/metabolic disturbances

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35
Q

What investigations might you need to investigate acute confusional states in SLE?

A

▪️LP to exclude infection
▪️EEG
▪️MRI

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36
Q

What MRI findings are commonly seen with acute confusional states in SLE?

A

White matter hyperintensities

37
Q

What CSF findings have been associated with acute confusional states in SLE?

A

High levels of:
▪️IL-6
▪️IgG index
▪️Anti-NR2
▪️Anti-Sm

38
Q

What are the main problems with understanding neuropsychiatric symptoms of lupus?

A

▪️Wide spectrum of features
▪️Poor understanding of pathogenesis
▪️Lack of diagnostic tools - largely clinical judgement
▪️Therapeutic approach is difficult

39
Q

How many neuropsychiatric syndromes have been identified in SLE?

A

19

40
Q

What are the six most common NP syndromes in SLE?

A

▪️Cognitive dysfunction
▪️Mood disorder
▪️Anxiety disorder
▪️Headache
▪️Seizure disorder
▪️Cerebrovascular disorder (Most commonly stroke, less commonly thrombosis)

41
Q

What are the four uncommon NP syndromes in SLE?

A

▪️Acute confusional state
▪️Psychosis
▪️Myelopathy
▪️Polyneuropathy

42
Q

What are some of the rare NP syndromes in SLE?

A

▪️Aseptic meningitis
▪️Demyelinating syndrome
▪️Guillain-Barre syndrome
▪️Autonomic neuropathy

43
Q

How does cognitive dysfunction usually present in SLE?

A

Commonly subtle and subclinical (e.g. Forgetful, brain fog, anomia, difficulties recognising people etc)

Occasionally will be severe and progressive

44
Q

How do you confirm cognitive dysfunction in SLE?

A

Formal psychometric testing, usually a 1 hour battery testing all domains

45
Q

What are the main problems with diagnosing common forms of NPSLE?

A

▪️Lack of specificity - symptoms common among non SLE patients too
▪️Hard to establish causality (e.g. CNS involvement or secondary functional features of chronic illness?)

46
Q

Psychosis is ___________ in SLE

A

Infrequent

Very low incidence!

47
Q

What else might cause psychosis in SLE?

A

Prolonged steroid use

48
Q

Why is it important to differentiate lupus psychosis from steroid induced psychosis?

A

▪️Lupus psychosis treated by increasing steroid dose/awaiting response
▪️If steroid induced, will need to reduce or stop prescription

49
Q

When are neuropsychiatric events most common in SLE?

A

Early on

50
Q

What percentage of NP manifestations in SLE are directly attributable to SLE?

A

20-30%

51
Q

What concomitant conditions may contribute to NP manifestations in SLE?

A

▪️Infection
▪️Hypertension
▪️Metabolic disturbance
▪️Toxic effect of therapy (e.g. steroids)

52
Q

If someone tests positive for the JC virus and is on aggressive immunosuppressant treatment for lupus, what are they at risk for?

A

Progressive multifocal leukoencephalopathy

(demyelinating lesions, neurological symptoms following treatment)

53
Q

What percentage of SLE patients will present with aseptic meningitis?

A

0.6% - very rare!

54
Q

What is aseptic meningitis?

A

Inflammation of the meninges that is NOT caused by bacterial infection

55
Q

What are the main signs of aseptic meningitis?

A

▪️Fever, headache
▪️Increased lymphocytes and immune cells in CSF but negative microbiology

56
Q

Why might SLE lead to aseptic meningitis?

A

▪️Active lupus
▪️Drug-induced (e.g. NSAIDs, immunosuppressants)

57
Q

What is PRES?

A

Posterior reversible encephalopathy syndrome

(vasogenic oedema caused by disruption of BBB)

(Rare NPSLE)

58
Q

What are the main signs of PRES?

A

▪️Altered mental state
▪️Cortical blindness
▪️Headache
▪️Seizures

59
Q

How can you spot PRES on MRI?

A

▪️Vasogenic oedema (extracellular fluid accumulation due to BBB disruption)
▪️Lesions typically in posterior occipital and parietal lobes

60
Q

What causes PRES in SLE?

A

▪️Secondary to autoimmunity
▪️Cytotoxic drugs
▪️Corticosteroids
▪️Hypertension

61
Q

What is the prognosis of PRES?

A

Usually good

Reversible with controlled arterial hypertension and step back from immunosuppressants

62
Q

What neurological complicationsay be seen with PRES?

A

▪️Endothelial dysfunction
▪️Vasodilation
▪️Hyperperfusion
▪️Haemorrhage

63
Q

What are the symptoms of idiopathic intracranial hypertension?

A

▪️Positional headache
▪️Nausea
▪️Vomiting
▪️Papilladema
▪️Visual disturbance
▪️6th cranial nerve palsy (eye movement)

64
Q

How do you treat idiopathic intracranial hypertension in SLE?

A

▪️Glucocorticoids
▪️Acetazolamide
▪️Mannitol

Good prognosis!

65
Q

How does idiopathic intracranial hypertension appear on brain imaging?

A

Normal

66
Q

How might you spot idiopathic intracranial hypertension with a lumbar puncture?

A

Normal CSF but high opening pressure

67
Q

What percentage of SLE patients present with PRES?

A

Less than 1%

68
Q

What percentage of SLE patients present with idiopathic intracranial hypertension?

A

1%

69
Q

How common is macrophage activation syndrome in SLE?

A

Quite rare but risen since covid due to viral infection

More common in children

70
Q

What is macrophage activation syndrome (MAS)?

A

▪️Hyperinflammagory condition
▪️Uncontrolled activation and proliferation of white blood cells
▪️Secondary inappropriate reaction to trigger, usually infection (haemophagocytic lymphohistiocytosis)

71
Q

What are the main symptoms of MAS?

A

▪️High fever
▪️Liver dysfunction and swelling
▪️Swelling of lymph nodes
▪️Deficiency of all blood cell types
▪️Increased iron stores and triglycerides
▪️Neurological abnormalities and altered mental status

72
Q

How is MAS treated and what are the problems?

A

▪️More challenging when it occurs with SLE
▪️Usually requires aggressive immunosuppression and ITU
▪️Aggressive neurosuppression if neurological abnormalities

73
Q

What are the 3 main proposed mechanisms for the pathogenesis of CNS SLE?

A

▪️Vascular abnormalities
▪️Local production of cytokines
▪️Direct effects of autoantibodies

74
Q

What NP symptoms may be indicative of APS?

A

Focal/thrombotic manifestations such as:
▪️Transient ischaemia attacks
▪️Ischaemic stroke
▪️Cerebral venous thrombosis

75
Q

If APS is suspected as the cause of NP symptoms, what can you do to confirm this?

A

Testing for antiphospholipid antibodies (aPL)

76
Q

What should you test for if a young person presents with a stroke (<50)?

A

Antiphospholipid syndrome (test for aPL)

77
Q

When can immunoglobulins be used?

A

In cases of severe CNS disease in the presence of severe infection so don’t want to use immunosuppression

78
Q

What is CSF analysis typically used for in NPSLE?

A

Excluding infection

79
Q

What is MRI typically used for in NPSLE?

A

Excluding other diseases

80
Q

What treatment is best for suspected thrombotic mechanism of NPSLE?

A

Antiplatelet or anticoagulant therapy

81
Q

What treatment is best for suspected inflammatory mechanism of NPSLE?

A

Corticosteroids/immunosuppressants

82
Q

What treatmemt should be used if someone is symptomatic and persistently positive for aPL?

A

Antiplatelet or anticoagulant therapy

83
Q

What treatment should be used for NPSLE if someone is symptomatic with evidence of active lupus?

A

Corticosteroids/immunosuppressants

84
Q

What antibodies are associated strongly with chorea and why?

A

aPL - binding in the basal ganglia?

85
Q

What antibodies are associated with seizures?

A

aPL

86
Q

How do you treat seizures in SLE?

A

▪️Antiepileptic treatment
▪️Consider steroids and immunosuppression if not improving or signs of CNS inflammation

87
Q

How do you treat myelitis in SLE/APS?

A

Anticoagulation and immunosuppression

ASAP to prevent permanent damage

88
Q

What can you use for refractory NPSLE?

A

Rituximab (RTX)

Management of Disorders (28 decks)

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