Systemic Lupus Erythematosus

Question 1

What is systemic lupus erythematosus?

Answer 1

Autoimmune rheumatic disease in which the body’s immune system mistakenly attacks healthy tissue, affecting many systems of the body.

Question 2

Describe the pathophysiology of SLE.

Answer 2

1. Genetic susceptibility combines with environmental triggers to cause apoptosis of cells.
2. Products of nuclear degradation are attacked by anti-nuclear antibodies due to decreased immune self-tolerance.
3. Antigen-antibody complexes form and cause local inflammation.

Question 3

Which type of hypersensitivity reactions occur in lupus?

Answer 3

Type 2 and 3 hypersensitivity.

Question 4

What is type 2 hypersensitivity?

Answer 4

Cytotoxic - the antibodies produced by the immune response bind to antigens on the patient’s own cell surfaces.

Question 5

What is type 3 hypersensitivity?

Answer 5

Immune complexes (antigen-antibody complexes) are formed and not cleared, causing an inflammatory response and attracting complement.

Question 6

Which group is SLE most common in? Why might this be?

Answer 6

Women of childbearing age 10:1. This may be due to the effect of estrogen.

Question 7

What are the diagnostic criteria for SLE?

Answer 7

4 out of 11 from: DOMAPIN(E)RASH - lecture
SKIN: Discoid rash
Oral ulcers
Photosensitivity
ARTHRITIS - symmetrical, >2 joints
Malar rash
IMMUNOLOGICAL disorder (eg anti-dsDNA)
NEURO - seizures, psychosis
(ESR raised but CRP normal)
RENAL - glomerulonephritis
ANA 
SEROSITIS - pericarditis/pleurisy
HAEMATOLOGICAL disorder (anaemia, thrombocytopaenia, leukopaenia)

Question 8

Which marker for SLE has high sensitivity and low specificity?

Answer 8

ANA

Question 9

Which marker for SLE has high specificity?

Answer 9

Anti-dsDNA

Question 10

What investigations would you do for suspected SLE and what would they show?

Answer 10

FBC: anaemia (of chronic disease/ autoimmune haemolytic)
ESR raised but CRP normal - very specific.
Auto-abs: Anti-DsDNA, ANA, anti-phospholipid
U&E: urea and creatinine raised in advanced disease
Biopsies - kidney, skin. Deposition of IgG and complement.

Question 11

Other than SLE, what could cause similar symptoms?

Answer 11

Infection
Other AI diseases
Malignancy
Endocrine disease
Metabolic disease

Question 12

Give 3 environmental triggers of SLE.

Answer 12

Drugs eg hydralazine
UV light
Viruses and bacteria

Question 13

Give 3 common features of autoimmune rheumatic diseases.

Answer 13

Pathology is inflammation causing scarring and damage in affected organs
Can affect any system and lead to organ failure
Auto-antibodies often present
Damage is irreversible
(lecture 1.2.18)

Question 14

Give 3 heritable ARDs.

Answer 14

Marfan’s syndrome
Ehler Danlos syndrome
Peyronie’s disease

Question 15

Give 3 autoimmune ARDs.

Answer 15

Rheumatoid arthritis
SLE
Sjögren’s syndrome

Question 16

What is anti-phospholipid syndrome?

Answer 16

Presence of anti-phospholipid antibodies with clinical features:
Coagulation defect (need anticoagulation)
Livedo reticularis
Obstetric (miscarriages - seek advice in pregnancy)
Thrombocytopaenia
-20-30% of people with SLE
-Can be primary.
(OHCM)

Question 17

What is lupus nephritis? How is it diagnosed?

Answer 17

Renal manifestation of SLE. Diagnosed clinically (hypertension, proteinuria, renal failure) and staged histologically using biopsy.

Question 18

How is lupus nephritis treated?

Answer 18

Class I/II: ACEi/ARB for renal protection, hydroxychloroquine (DMARD) for extra-renal disease.
Class III-IV: immunosuppresion - glucocorticoids, cyclophosphamide, rituximab

Question 19

Describe the non-pharmacological management of SLE.

Answer 19

1. Educate and support - UV protection
2. Assessment of disease activity - clinical and immunological, screen for co-morbidities
3. Refer - complex cases should involve specialist SLE/nephritis clinics.

Question 20

Give 3 drugs which can cause SLE.

Answer 20

Anti-TNF agents
Procainamide (anti-arrhythmic)
Hydralazine (anti-hypertensive)

Question 21

Describe the pharmacological management of SLE.

Answer 21

Immunosuppressive:
Hydroxychloroquine (DMARD) 
Azathioprine, methotrexate
Belimumab if disease activity is high
Symptomatic:
Antidepressants (high rate mental health issues)
NSAIDs unless renal disease
Reduce CV risk
Bisphosphonates for OP

Question 22

How are skin flares treated in SLE?

Answer 22

Topical steroids

Question 23

How are mild and moderate flares treated in SLE?

Answer 23

If no organ involvement, hydroxychloroquine or steroids

If organ involvement, DMARDS/mycophenolate.

Question 24

How are severe flares treated in SLE?

Answer 24

Urgent high-dose steroids, rituximab, cyclophosphamide.

MDT vital