Systemic Lupus Erythematosus Flashcards
More specific tests for SLE include
● 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
Malar rash
Fixed erythema, flat or raised, over the cheeks, tending to spare the nasolabial folds
Discoid rash
Erythematous raised patches with adherent keratotic scaling and follicular plugging (atrophic scarring can occur in older lesions)
Photosensitivity
Skin rash caused by an unusual reaction to sunlight (determined by patient history or clinician observation)
Oral ulcers
Oral or nasopharyngeal ulceration, usually painless
Arthritis
Non-erosive arthritis involving at least two peripheral joints, characterised by tenderness, swelling or effusion
Pleuritis or pericarditis
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)
Renal disorder
Either persistent proteinuria or cellular casts in the urine (eg, from red blood cells or haemoglobin)
Neurological disorder
Either seizures or psychosis (that have no other precipitating causes, such as drugs or metabolic derangements)
Haematological disorder
Either haemolytic anaemia, leucopenia or thrombocytopenia
Immunological disorder
Presence of antibodies to double-stranded DNA (anti-dsDNA) or anti-Sm antibodies or a positive finding of
antiphospholipid antibodies
Antinuclear antibody
Raised titre of antinuclear antibody in the absence of drugs known to be associated with drug-induced lupus
The goals of therapy for patients with SLE are to:
● 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
Management of SLE in pregnancy
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.
Antiphospholipid syndrome
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
Pharmacological management
- NSAIDs
- Antimalarials (Hydroxychloroquine)
- Corticosteroids
- Immunomodulators (Azathioprine, methotrexate, leflunomide,
mycophenolate and cyclophosphamide) - Biological therapy (Belimumab, Rituximab )
Mild activity/flare - Initial typical drugs and target doses if no contra-indications
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)
Moderate activity/flare - Initial typical drugs and target doses if no contra-indications
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
Severe activity/flare - Initial typical drugs and target doses if no contra-indications
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
Mild activity/flare - typical maintenance drugs/doses providing no contra-indications
Prednisolone ≤ 7.5 mg/day
and HCQ 200 mg/day
and/or MTX 10 mg/week
Moderate activity/flare - typical maintenance drugs/doses providing no contra-indications
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
Severe activity/flare - typical maintenance drugs/doses providing no contra-indications
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;
How to stop therapy
Aim to reduce and stop drugs except HCQ eventually when in stable remission
Diagnostic tests
ANA Anti-S-DNA antibodies aPLs Igs ANCA Anti Ro
