Scoring Systems for Assessing Disease Activity in Systemic Lupus Erythematosus (SLE)
Disease activity in SLE should be assessed using validated indices at each clinical visit, with SLEDAI-2K and SLE-DAS being the most widely used and validated scoring systems. 1
Primary Validated Disease Activity Indices
SLEDAI-2K (SLE Disease Activity Index 2000)
- Most commonly used standardized scoring system
- Evaluates disease activity over a 30-day period
- Covers 9 organ systems with 24 descriptors
- Score range: 0-105 (higher scores indicate greater disease activity) 2, 3
- Advantages:
- Well-validated and widely accepted
- Predicts mortality
- Relatively quick to complete
- Disadvantages:
- Binary scoring (present/absent) for manifestations
- May not capture improving but persistent disease activity
SLE-DAS (SLE Disease Activity Score)
- Newer continuous scoring system introduced in 2019
- Validated cut-off of 6.62 for defining Low Lupus Disease Activity State (LLDAS) 4
- Advantages:
- Disadvantages:
- Less widely implemented in clinical practice
- Newer with less long-term validation data
BILAG (British Isles Lupus Assessment Group)
- Organ-based scoring system
- Categorizes disease activity by organ system (A-E grading)
- Advantages:
- Detailed assessment of individual organ systems
- Captures improvement and worsening
- Disadvantages:
- More complex and time-consuming to complete 6
Other Validated Indices
- ECLAM (European Consensus Lupus Activity Measurement)
- SLAM (Systemic Lupus Activity Measure)
- LAI (Lupus Activity Index) 6, 7
Comprehensive Assessment Framework
According to EULAR recommendations, SLE assessment should include 1:
Disease activity measurement using a validated index at each visit
- SLEDAI-2K or SLE-DAS are preferred options
Organ damage assessment annually
- Using SLICC/ACR damage index (SDI)
Quality of life evaluation at each visit
- Patient history or 0-10 visual analog scale (patient global score)
Laboratory monitoring
- Anti-dsDNA antibodies and complement (C3, C4) levels
- Complete blood count
- Erythrocyte sedimentation rate
- C-reactive protein
- Serum albumin
- Serum creatinine (or eGFR)
- Urinalysis and urine protein/creatinine ratio
Specific Organ System Assessment
Mucocutaneous Involvement
- Use validated indices like CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index) 1
- Characterize lesions as:
- LE specific
- LE non-specific
- LE mimickers
- Drug-related
Renal Involvement
- Monitor protein/creatinine ratio (or 24h proteinuria)
- Immunological tests (C3, C4, anti-dsDNA)
- Urine microscopy and blood pressure at least every 3 months for first 2-3 years 1
Neuropsychiatric Manifestations
- Monitor through focused history for neuropsychological symptoms
- Assess cognitive impairment by evaluating attention, concentration, word finding, and memory difficulties 1
Clinical Implementation
Use disease activity scores to guide treatment decisions and define treatment targets
LLDAS (Low Lupus Disease Activity State) is a validated treatment target:
For research and clinical trials, composite indices are often used:
- SRI (SLE Responder Index): combines SELENA-SLEDAI, PGA, and BILAG
- BICLA (BILAG-Based Composite Lupus Assessment) 1
Common Pitfalls and Caveats
- Distinguish disease activity from damage, infection, or drug toxicity
- Laboratory markers (anti-dsDNA, complement) may not always correlate with clinical activity
- Some manifestations (e.g., fatigue) may not be captured adequately by activity indices
- Regular reassessment is essential as SLE has a relapsing-remitting course
- Scoring systems should guide but not replace clinical judgment
By implementing standardized disease activity assessment using validated indices like SLEDAI-2K or SLE-DAS at each clinical visit, clinicians can better monitor disease progression, guide treatment decisions, and improve outcomes for patients with SLE.