Systemic Lupus Erythematosus

Question 1

More specific tests for SLE include

Answer 1

● Antibodies to double-stranded DNA (known as anti-dsDNA), which are present in 70% of patients with SLE
● Anti-Sm antibodies, which are present in 30–40% of patients with SLE

Question 2

Malar rash

Answer 2

Fixed erythema, flat or raised, over the cheeks, tending to spare the nasolabial folds

Question 3

Discoid rash

Answer 3

Erythematous raised patches with adherent keratotic scaling and follicular plugging (atrophic scarring can occur in older lesions)

Question 4

Photosensitivity

Answer 4

Skin rash caused by an unusual reaction to sunlight (determined by patient history or clinician observation)

Question 5

Oral ulcers

Answer 5

Oral or nasopharyngeal ulceration, usually painless

Question 6

Arthritis

Answer 6

Non-erosive arthritis involving at least two peripheral joints, characterised by tenderness, swelling or effusion

Question 7

Pleuritis or pericarditis

Answer 7

Either pleuritis (convincing history of pleuritic pain or clinical evidence of pleural effusion) or pericarditis (diagnosed using electrocardiogram or through clinical evidence of pericardial effusion)

Question 8

Renal disorder

Answer 8

Either persistent proteinuria or cellular casts in the urine (eg, from red blood cells or haemoglobin)

Question 9

Neurological disorder

Answer 9

Either seizures or psychosis (that have no other precipitating causes, such as drugs or metabolic derangements)

Question 10

Haematological disorder

Answer 10

Either haemolytic anaemia, leucopenia or thrombocytopenia

Question 11

Immunological disorder

Answer 11

Presence of antibodies to double-stranded DNA (anti-dsDNA) or anti-Sm antibodies or a positive finding of
antiphospholipid antibodies

Question 12

Antinuclear antibody

Answer 12

Raised titre of antinuclear antibody in the absence of drugs known to be associated with drug-induced lupus

Question 13

The goals of therapy for patients with SLE are to:

Answer 13

● Control symptoms, such as joint pain and fatigue
● Prevent flares or treat them when they occur
● Minimise damage to organs
● Avoid long-term complications from the medicines used

Question 14

Management of SLE in pregnancy

Answer 14

Where possible, cyclophosphamide, methotrexate and mycophenolate should be discontinued (and disease should be clinically stable on an alternative therapy that is safe in pregnancy) six to 12 months before conception.

Antimalarials, corticosteroids and azathioprine can be
continued throughout pregnancy.

Question 15

Antiphospholipid syndrome

Answer 15

Treatment with hydroxychloroquine, with or without aspirin, can be considered for patients with SLE and antiphospholipid antibodies, to prevent thrombotic events.

Warfarin therapy is recommended (with a target international normalised ratio of 2–3) for patients with antiphospholipid antibodies and a history of venous thromboembolism; long-term anticoagulation is generally
recommended because the rate of thrombus recurrence is up to 70% when anticoagulation is stopped.9 For pregnant women low molecular weight heparin is preferred

Question 16

Pharmacological management

Answer 16

• NSAIDs
• Antimalarials (Hydroxychloroquine)
• Corticosteroids
• Immunomodulators (Azathioprine, methotrexate, leflunomide,
  mycophenolate and cyclophosphamide)
• Biological therapy (Belimumab, Rituximab )

Question 17

Mild activity/flare - Initial typical drugs and target doses if no contra-indications

Answer 17

CSs: Topical preferred or oral prednisolone ≤20 mg daily for 1–2 weeks
or I.m. or IA methyl-prednisolone 80–120 mg

and HCQ ≤6.5 mg/kg/day
and/or MTX 7.5–15 mg/week
and/or NSAIDs (for days to few weeks only)

Question 18

Moderate activity/flare - Initial typical drugs and target doses if no contra-indications

Answer 18

Prednisolone ≤0.5 mg/day
or i.v. methyl-prednisolone ≤250 mg × 1–3
or i.m. methyl-prednisolone 80–120 mg

and AZA 1.5–2.0 mg/kg/day or MTX (10–25 mg/week) or MMF (2–3 g/day) or ciclosporin ≤2.0 mg/kg/day
and HCQ ≤6.5 mg/kg/day

Question 19

Severe activity/flare - Initial typical drugs and target doses if no contra-indications

Answer 19

Prednisolone ≤0.5 mg/day
and/or i.v. methyl-prednisolone 500 mg × 1–3
or prednisolone ≤0.75–1 mg/kg/day

and AZA 2–3 mg/kg/day or MMF 2–3 g/day or CYC i.v. or ciclosporin ≤2.5 mg/kg/day
and HCQ ≤6.5mg/kg/day

Question 20

Mild activity/flare - typical maintenance drugs/doses providing no contra-indications

Answer 20

Prednisolone ≤ 7.5 mg/day

and HCQ 200 mg/day
and/or MTX 10 mg/week

Question 21

Moderate activity/flare - typical maintenance drugs/doses providing no contra-indications

Answer 21

Prednisolone ≤ 7.5 mg/day

and AZA 50–100 mg/day
or MTX 10 mg/week
or MMF 1 g/day
or ciclosporin 50–100 mg/day

Question 22

Severe activity/flare - typical maintenance drugs/doses providing no contra-indications

Answer 22

Prednisolone ≤ 7.5 mg/day

and MMF 1.0–1.5 g/day
or AZA 50–100 mg/day
or ciclosporin 50–100 mg/day
and HCQ 200 mg/day;

Question 23

How to stop therapy

Answer 23

Aim to reduce and stop drugs except HCQ eventually when in stable remission

Question 24

Diagnostic tests

Answer 24

ANA
Anti-S-DNA antibodies
aPLs
Igs
ANCA
Anti Ro

Question 25

What is SLE

Answer 25

Systemic lupus erythematosus or lupus as it is more commonly known is a complex autoimmune disease that attacks a variety of organs

Question 26

Possible causes and contributory factors

Answer 26

• Genetic and environmental factors
• UV light
• Vitamin D deficiency
• Female sex hormones particularly oestrogen
• Drug induced e.g. hydralazine, procainamide

Question 27

The ‘classical’ presentation of SLE

Answer 27

involves a woman of child bearing age presenting with a butterfly facial rash, mouth ulcers and alopecia

Question 28

Other symptoms of lupus

Answer 28

photosensitive (discoid) skin rashes, pleuritis, headaches,

depression, anxiety and Raynaud’s phenomenon.

Question 29

Sjögren’s syndrome involves

Answer 29

the lacrimal ducts which are attacked

via an autoimmune mechanism, resulting in a condition involving dry eyes and dry mouth.

Question 30

Antiphospholipid syndrome significantly increases

Answer 30

the risk of patients having a thrombotic
event e.g. DVT or PE. Young women with a history of recurrent miscarriage may have
antiphospholipid syndrome.

Question 31

Pharmacological management categories

Answer 31

• Induction – medicines used during a flare to induce remission.
• Maintenance – medicines used to consolidate remission and reduce the risk of further flares.

Question 32

three major complications associated with SLE

Answer 32

• Sjögren’s Syndrome
• Antiphospholipid Syndrome
• Raynaud’s Phenomenon

Question 33

Raynaud’s Phenomenon caused by

Answer 33

This is caused by spasm of the peripheral arterioles, symptoms include cold hands and feet which may turn blue in cold weather. When blood returns to the extremities it can be extremely painful. Treatment involves keeping the extremities warm and wearing gloves.
In more severe cases nifedipine may be used for its peripheral vasodilatory properties.

Question 34

SLE and pregnancy

Answer 34

Pregnant women who have SLE, have an increased risk of disease flares, miscarriage, thrombosis, pre-eclampsia and preterm delivery. Careful discussion between female
patients with SLE (regardless of whether they have antiphospholipid syndrome) and a specialist clinician should take place ideally, before conception.