Systemic Lupus Erythematosus (SLE)
Description Diagnostic criteria General and supportive measures Medicine treatment Referral ICD-10 code: M32.9 DESCRIPTION Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease characterised by the presence of auto-antibodies directed against various cellular components, particularly DNA. It is often associated with antiphospholipid-antibodymediated hypercoagulability. In
Description
Diagnostic criteria
General and supportive measures
Medicine treatment
Referral
ICD-10 code: M32.9
DESCRIPTION
Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease characterised by the presence of auto-antibodies directed against various cellular components, particularly DNA. It is often associated with antiphospholipid-antibodymediated hypercoagulability. In children it predominantly targets the kidneys (in 50– 80%), central nervous system, skin and joints.
Treatment of acute lupus depends on severity of illness, with more aggressive treatment for CNS, renal and haematologic involvement.
DIAGNOSTIC CRITERIA
Clinical
Diagnosis may be elusive due to its variations in presentation and is confirmed with: SLICC CLASSIFICATION FOR SLE.
Requirements: ≥ 4 criteria (at least 1 clinical and 1 laboratory criteria) OR biopsyproven lupus nephritis with positive ANA or anti-double stranded DNA antibody.
| CLINICAL CRITERIA | IMMUNOLOGIC CRITERIA (positive result) |
| 1. Acute Cutaneous Lupus 2. Chronic Cutaneous Lupus 3. Oral or nasal ulcers 4. Non-scarring alopecia 5. Arthritis 6. Serositis 7. Renal 8. Neurologic 9. Haemolytic anaemia 10. Leukopenia 11. Thrombocytopenia (<100 000/mm3 ) |
1. ANA
|
Investigations
Note: Normal urine analysis does not exclude renal disease.
- Urine test strip: haematuria and proteinuria.
- Urine microscopy: cellular casts.
- FBC: differential and platelet count.
- Complement, anti-nuclear antibodies, anti-double stranded DNA antibodies.
- Screen for thyroid involvement.
- Serum urea, creatinine, electrolytes, albumin and cholesterol.
- Clotting profile, antiphospholipid antibody and lupus anticoagulant.
- Electrocardiography and chest X-ray.
GENERAL AND SUPPORTIVE MEASURES
- Counselling, education and a team approach.
- Adequate rest and appropriate nutrition.
- Protect from sunlight: use sunscreen and hats; avoid sunlight if unprotected.
- Physiotherapy to relieve arthralgia.
- Psychological support.
- Immunisation, especially pneumococcal vaccine.
- Prompt management of infections.
MEDICINE TREATMENT
All children should be treated by a specialist.
- Vitamin D and calcium supplementation.
All children:
- Chloroquine (as base), oral, 5 mg/kg/dose daily, Monday to Friday.
- Maximum dose: 200 mg.
- 6-monthly eye examination necessary.
Chloroquine has a disease-modifying role and is particularly useful for skin and joint disease; some patients can be managed with chloroquine alone or with the addition of low-dose steroids.
Induction therapy
The options depend on the severity of the disease and major organ involvement.
For general systemic disease, serositis or musculoskeletal disease:
- Corticosteroid treatment:
- Prednisone, oral 2 mg/kg/day; maximum daily dose 60 mg.
- Reduce dose to 0.5 mg/kg once daily by 2 months.
For major organ involvement (severe lupus nephritis class III or IV and neuropsychiatric lupus):
- Methylprednisolone, IV, 30 mg/kg/day (maximum 1000 mg) for 3 days followed by oral prednisone 2 mg/kg/day.
- Reduce dose to 0.5 mg/kg once daily by 2 months.
AND
- Cyclophosphamide, IV, 500–750 mg/m2 /dose, administered over 2 hours.
- Repeat once a month for 6 months.
- Cyclophosphamide must be given with pre-hydration and continue increased fluid intake for 24 hours after cyclophosphamide infusion.
- Monitor vital signs during administration of cyclophosphamide.
Maintenance treatment (steroid sparing treatment)
For mild/moderate disease (vasculitic rash, cytopenia, serositis):
- Azathioprine, oral, 1–2.5 mg/kg/dose as a single daily dose.
- Maximum dose: 150 mg.
- Refer if contraindication to azathioprine or if patient develops adverse effects with treatment.
For musculoskeletal and skin disease:
- Methotrexate, oral, 10–15 mg/m2 /week as a single dose on an empty stomach. Specialist initiated.
- Maximum dose: 25 mg/week.
PLUS
- Folic acid, oral, 5 mg weekly (on the day after methotrexate) for the duration of the treatment.
REFERRAL
Specialist referral:
- All patients for confirmation of diagnosis and initiation/supervision of treatment.
- All patients receiving chloroquine treatment must be referred for ophthalmologic examination.
- Macrophage activation syndrome.
- For kidney biopsy if any evidence of renal disease (deteriorating renal function, significant proteinuria/haematuria or hypertension).
Standard Treatment Guidelines And Essential Medicines List For South Africa, Hospital Level, Paediatrics, 2023 Edition
Chapter 12: Rheumatology And Vasculitides, 12.4 Systemic Lupus Erythematosus (SLE)