Lupus

Question 1

What is the immunological pathogenesis of lupus?

Answer 1

Innate immunity is under active, failing to clear apoptotic material
Adaptive response is triggered to clear nuclear material still in circulation
Antibodies are developed against self material

Question 2

What systems are affected by lupus?

Answer 2

Mucocutaneous
Nephritis
Neuropsychiatric
Musculoskeletal
Cardiopulmonary
Haematological

Question 3

What’s the m:f ratio of lupus?

Answer 3

9:1 female

Question 4

What demographics have a poorer outcome?

Answer 4

Young
Black (more severe disease phenotype)

Question 5

What is the prevalence of lupus?

Answer 5

1 in 1000

Question 6

What is the 15 year survival rate of lupus?

Answer 6

84% (1 in 6 chance of dying)

Question 7

What is the most common sequela of lupus?

Answer 7

Lupus nephritis - 50%

Question 8

What is the standard treatment for lupus?

Answer 8

Hydroxychloroquine - (takes 6 months to work, is well tolerated)

Question 9

What is used for an acute flare of lupus?

Answer 9

Glucocorticoids eg prednisolone

Question 10

What steroid sparing medications may be used?

Answer 10

Mycophenolate - renal
Azathioprine - lupus management in pregnancy
Methotrexate - joint and skin

Question 11

What is the top line treatment for severe lupus?

Answer 11

Rituximab (anti-B cell antibody)
Cyclophosphamide (chemotherapy)

Question 12

Given the demographic, what is a primary concern with cyclophosphamide?

Answer 12

Fertility

Question 13

What is the age of onset of lupus?

Answer 13

Child bearing years
12-50

Question 14

What is a neuropsychiatric sequela of steroid use?

Answer 14

Mania/hypomania

Question 15

What scale is a measure of severity of lupus?

Answer 15

SLEDAI-2K

Question 16

What are examples of non compliance in lupus?

Answer 16

1/3 don’t take medication
Many don’t come into clinic
(Due to issues of executive function)

Question 17

What conditions fall under neuropsychiatric lupus?

Answer 17

Wide ranging
CNS and PNS

Stroke
Demyelinating syndrome + myelopathy
Headache
Movement disorder
Seizure
Acute confessional state
Mood disorder
Cognitive dysfunction
Psychosis
Neuropathies/plexopathies
Myasthenia gravis

Question 18

What are the three factors for neuropsychiatric lupus?

Answer 18

Immunosuppression (corticosteroids/cyclophosphamide/rituximab)

Antipsychotics

Time

Question 19

What are possible causes of neuropsychiatric symptoms of lupus?

Answer 19

Direct effects of inflammation
Damage from previously active lupus (eg stroke damage)
Iatrogenic effects of treatment
Concurrent psychiatric disorder (related or unrelated to SLE)

Question 20

What was the patient reported incidence of hallucinations?

Answer 20

1 in 4

Question 21

What are other commmonly reported symptoms?

Answer 21

Fatigue
Loss of coordination
Suicidal thoughts
Cognitive dysfunction
Positive and negative sensory symptoms
Mania
Low mood

Question 22

What is a possible serological biomarker of neuropsychiatric lupus?

Answer 22

Anti-ribosomal P antibody

Non-specific to the brain symptoms - does not correlate to CSF levels either

Question 23

What is the utility of CSF to identify neuropsychiatric lupus?

Answer 23

No specific biomarkers outside of inflammation

Question 24

What is the utility of imaging to identify neuropsychiatric lupus?

Answer 24

No biomarkers as yet
Potential target could be BBB permeability (hippocampus)

Question 25

What may be seen in lupus neuroimaging?

Answer 25

White matter changes