From the Guidelines
A flare in Pediatric Systemic Lupus Erythematosus (SLE) is defined as a measurable increase in disease activity, usually leading to a change of treatment.
Flare Criteria
The exact criteria for a flare in pediatric SLE are not universally agreed upon, but most experts agree that it involves an increase in disease activity that necessitates a change in treatment 1.
- Risk factors for a higher disease flare rate include:
- Younger age at disease onset
- No use of antimalarials
- Persistent generalized disease activity
- Serological activity (anti-dsDNA, low complement)
Management
In pediatric SLE, kidney involvement is more common and often presents as a manifestation of the disease, with renal flares observed in over 50% of patients 1.
- Management of pediatric lupus nephritis (LN) should follow the same principles as in adult disease, with consideration of pediatric doses of drugs.
- A transition program is recommended for adolescents to ensure adherence and optimal outcomes.
- Close monitoring and optimization of disease control may reduce the risk of a flare, particularly in patients with risk factors 1.
From the Research
Flare Criteria for Pediatric Systemic Lupus Erythematosus (SLE)
The flare criteria for Pediatric Systemic Lupus Erythematosus (SLE) have been established through various studies, including those published in 2018 2, 2011 3, and 2010 4. The criteria are based on the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) scores, as well as other factors such as physician global assessment of disease activity, protein/creatinine ratio, and erythrocyte sedimentation rate.
SLEDAI-Based Criteria
- Flare scores ≥6.4/3.0/0.6 constitute major/moderate/minor flares, respectively, based on the SLEDAI-based criteria (0.5 × ∆SLEDAI + 0.45 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) 2
- These threshold values were found to be >82% sensitive and specific for capturing flare severity 2
BILAG-Based Criteria
- Flare scores ≥7.4/3.7/2.2 delineate major/moderate/minor flares, respectively, based on the BILAG-based algorithm (0.4 × ∆BILAG + 0.65 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) 2
- These threshold values were also found to be >82% sensitive and specific for capturing flare severity 2
Other Studies
- A 2011 study found that flare scores can be calculated using the formula (0.5 × ΔSLEDAI + 0.45 × ΔP:C ratio + 0.5 × ΔMD-global + 0.02 × ΔESR), where values of ≥1.04 are reflective of a flare 3
- A 2010 study found that a definition of global flare in juvenile SLE is "a measurable worsening of juvenile SLE disease activity in at least one organ system, involving new or worse signs of disease that may be accompanied by new or worse SLE symptoms" 4
- A 2025 study validated novel childhood-onset SLE targets, including Childhood Lupus Low Disease Activity State (cLLDAS), cSLE Clinical Remission on steroids (cCR), and cSLE Clinical Remission off steroids (cCR-0) 5
- A 2018 study verified the sensitivity and specificity of the SLICC classification criteria for juvenile systemic lupus erythematosus, finding higher sensitivity and accuracy using the SLICC criteria compared to the ACR lupus criteria 6