Definition and epidemiology
Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease driven by autoantibody production, immune-complex deposition, and complement activation. Childhood-onset SLE (cSLE) accounts for roughly 15–20% of all lupus and is more aggressive than adult disease, with higher rates of renal and neuropsychiatric involvement and greater cumulative organ damage. Onset is uncommon before age 5; incidence rises through adolescence with a strong female predominance that increases after puberty. In India, cSLE is well recognised, often presenting late with active nephritis and infection as a major competing cause of morbidity.
Clinical features
Presentation is protean. Common features include constitutional symptoms (fever, fatigue, weight loss), a photosensitive malar (butterfly) rash, oral or nasal ulcers, non-scarring alopecia, and non-erosive arthritis. Serositis (pleuritis, pericarditis), Raynaud phenomenon, and cytopenias (haemolytic anaemia, leukopenia, thrombocytopenia) are frequent. Lupus nephritis is the single most prognostically important manifestation in children and may be clinically silent until proteinuria, hypertension, or rising creatinine appear — a low threshold for urinalysis is essential. Neuropsychiatric lupus (seizures, psychosis, cognitive change) and antiphospholipid-associated thrombosis carry high morbidity.
Diagnosis
SLE is a clinical diagnosis supported by classification criteria. The 2019 EULAR/ACR criteria require a positive ANA at a titre ≥1:80 as an obligatory entry criterion; without it, a patient is not classified. Thereafter, additive weighted criteria across seven clinical domains (constitutional, haematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunologic domains (antiphospholipid antibodies, complement, SLE-specific antibodies — anti-dsDNA/anti-Smith) are scored, counting only the highest-weighted item per domain. A score of ≥10 with at least one clinical criterion classifies SLE (sensitivity ~96%, specificity ~93%). Criteria should not be counted if a more likely explanation exists. The older SLICC 2012 criteria remain a useful alternative. These are classification (research) criteria, not a diagnostic rule-out — sensitivity is somewhat lower in childhood-onset cohorts, so an ANA-negative child with otherwise convincing lupus still warrants clinical judgement. Workup includes CBC, creatinine, urinalysis with protein quantification, C3/C4, anti-dsDNA, anti-Sm, antiphospholipid antibodies, and Coombs test.
See the full criteria: Systemic Lupus Erythematosus (ACR)
Red flags
- New or worsening proteinuria, hypertension, or rising creatinine — suspect active lupus nephritis; expedite renal evaluation and biopsy.
- Fever in a lupus patient — distinguish flare from infection; immunosuppression and complement deficiency raise sepsis risk markedly.
- Seizures, psychosis, or acute confusion — neuropsychiatric lupus or alternative CNS pathology.
- Cytopenias with fever and very high ferritin — consider macrophage activation syndrome (HLH), a lupus-associated emergency.
- Acute chest pain or dyspnoea — pericardial tamponade, pulmonary haemorrhage, or thrombosis.
Management overview
Management is risk-stratified and best co-managed with paediatric rheumatology and nephrology. Hydroxychloroquine is the backbone for nearly all patients (reduces flares, protects organs, improves survival). Glucocorticoids control active disease but should be tapered to the lowest effective dose to limit growth and bone toxicity. Major organ involvement (proliferative nephritis, CNS disease) requires immunosuppression — mycophenolate mofetil or cyclophosphamide for induction, with mycophenolate or azathioprine for maintenance; belimumab and rituximab are options in refractory disease. Essential supportive care: photoprotection, blood-pressure control with ACE inhibitors in proteinuric nephritis, vaccination (avoid live vaccines on heavy immunosuppression), bone health, and aggressive infection vigilance. Monitor anti-dsDNA and complement as activity markers, and screen for damage accrual.
References
- Aringer M, et al. 2019 EULAR/ACR Classification Criteria for SLE. Arthritis Rheumatol. 2019;71(9):1400-1412.
- Petri M, et al. SLICC classification criteria for SLE. Arthritis Rheum. 2012;64(8):2677-2686.
- IAP Rheumatology Chapter — childhood SLE consensus guidelines.
- Nelson Textbook of Pediatrics, 21st ed.
Decision support for qualified clinicians only — verify against current primary guidelines and your clinical judgement.