Table 1

Features, caveats and pitfalls of main indices used in SLE: the SLEDAI-2K, the SELENA-SLEDAI Flare Index and the SLICC/ACR Damage Index

IndexFeatures and clinical relevanceHow to use, caveats and pitfalls
  • Scores the activity of 24 clinical presentations within a period of 28 days

  • Organ involvement is weighted from 1 to 8 (range 0–105)

Grading of severity
  • SLEDAI=0 Remission

  • SLEDAI=1–4 Low activity

  • SLEDAI=5–10 Moderate activity

  • SLEDAI >10 High activity

Clinically important changes
  • Increase >3 = Flare

  • Decrease <3 = Improvement

  • Change ±3 = Persistent activity

  • Combine SLEDAI with a Physician Global Assessment (PGA) (graded from 0 to 3 on a 10 cm long straight line)

  • Assess PGA before calculating the SLEDAI, to avoid bias in physician assessment

  • Score items only if confidently attributed to lupus

    Pitfalls: pyuria due to UTI or asymptomatic bacteriuria; hair loss or leucopenia due to drug side effect; stroke due to atherosclerosis; other neuropsychiatric manifestations due to metabolic abnormalities, drug side effects or CNS infections

  • Score items only if they are reversible

    Pitfalls: scarring alopecia; ‘fixed’ lupus rash with scar; ‘fixed proteinuria’

  • Time needed to complete: 5–10 min

SELENA-SLEDAI Flare indexFlares defined by:
  • changes in SLEDAI score and/or individual manifestations and/or changes in treatment and/or need for hospitalisation and/or changes in PGA

Mild/moderate flare
  • Change in SELENA-SLEDAI instrument score of 3 points or more (but not to >12)

  • Increase in prednisone, but not to >0.5 mg/kg/day

  • Addition of NSAID, hydroxychloroquine for SLE activity

  • ≥ 1.0 increase in PGA score, but not to >2.5

Severe flare
  • Change in SELENA-SLEDAI instrument score to >12 points

  • Increase in prednisone to >0.5 mg/kg/day

  • New cyclophosphamide, azathioprine, methotrexate, MMF or biologics for SLE activity

  • Hospitalisation for SLE

  • Increase in PGA score to >2.5

  • Patients classify for flare if ≥1 criterion for flare is present

  • Treatment changes qualify for a flare, even in case of persistent activity rather than exacerbation

  • A treatment change does not always correlate with physician assessment of disease activity

  • A ‘major flare’ can result from small increases in disease activity from different domains

  • No discrimination between mild vs moderate flares

  • Both number and severity of flares have been associated with irreversible damage accrual (SDI increase)

  • Time needed to complete: 10–20 min

  • Scores irreversible damage accrual in 12 organ systems

  • Damage due to either disease or medication side-effects (eg, glucocorticoids or cyclophosphamide)

Grading of damage
  • SDI 0 No damage

  • SDI≥1 Irreversible damage present

  • SDI≥3 Severe damage present

Clinical relevance
  • Any increment in the SDI is prognostically significant, associated with further damage accrual and mortality

  • Score damage occurring only after SLE onset

  • Score items present for at least 6 months (beware for potentially reversible manifestations, eg, proteinuria, alopecia)

  • Since damage items are irreversible, SDI can only increase over time (unlike eg, the Health Assessment Questionnaire in RA)

  • Individual items get same score if present, irrespective of extent of damage and impact on patient’s life

    Examples: Stroke with minimal neurologic sequelae vs severe neurologic deficit; pulmonary fibrosis limited vs extensive

  • Time needed to complete: 10–20 min

  • ACR, American College of Rheumatology; CNS, central nervous system; MMF, mycophenolate mofetil; NSAID, non-steroidal anti-inflammatory drugs; RA, rheumatoid arthritis; SDI, SLICC/ACR Damage Index; SELENA, Safety of Estrogens in Lupus Erythematosus National Assessment; SLE, systemic lupus erythematosus ; SLEDAI, SLE Disease Activity Index; SLICC, Systemic Lupus International Collaborating Clinics; UTI, urinary tract infection.