Background/Purpose:  The heterogeneity of SLE in terms of disease characteristics is an issue not only for the diagnostic process but also for disease management. Previously, a SLEDAI score ≥10, herein termed the High Disease Activity State (HDAS), has been shown to predict treatment response to belimumab. Whether HDAS could provide other prognostic information on outcomes is less well understood.

Methods:  Using data collected prospectively between 2007 and 2015 from 211 SLE patients, who fulfilled either ACR (97.2%) or only SLICC (2.8%) classification criteria and were seen at a tertiary SLE clinic for ≥1 year, we examined the association of sociodemographic and disease characteristics with ever experiencing HDAS during the observed period. We also investigated the association of ever experiencing HDAS with longitudinal SLE outcomes, including adjusted mean SLEDAI (AMS), flare incidence according to SLE Flare Index, and damage accrual according to the SLICC/ACR Damage Index for SLE. For multivariable analyses of longitudinal outcomes, associations were adjusted for observation time, and the association with AMS was also adjusted for cumulative prednisolone dose.

Results:  Patients were observed for a median of 4.5 years (range: 1 – 7.9 years); 42.7% experienced HDAS at least once during the observation period. Among those who experienced HDAS, 31.1% experienced a single episode of HDAS, 33.3% experienced 2 – 3 episodes and the remainder experienced between 4 – 30 episodes of HDAS. The median time to first HDAS was 9 months after enrolment (range 0 – 6.7 years). Females patients diagnosed at ≥45 years had substantially lower odds of ever experiencing HDAS (Odds Ratio, OR: 0.3, 95% Confidence Interval, 95% CI: 0.1 – 0.5; p=0.003). When compared to patients never experiencing HDAS, patients experiencing at least 1 episode of HDAS were more likely to have anti-dsDNA autoantibodies (OR: 5.6, 95%CI: 2.8 – 11.3; p<0.001), have a positive direct antiglobulin test (OR: 2.7, 95%CI: 1.3 – 5.3; p = 0.006) or present with hypocomplementemia (OR: 2.3, 95% CI: 1.3 – 4.0; p=0.005). Patients who experienced at least one episode of HDAS had significantly greater odds of having AMS in the highest quartile (AMS ≥4.3: OR: 10.8, 95%CI: 4.3 – 27.2; p<0.001). Similarly, they were also more likely to experience a greater number of mild/moderate and severe flares during the observation period (number of mild/moderate flares ≥5: OR: 8.0, 95% CI: 3.1 – 21.1; p<0.001; number of severe flares ≥3: OR: 16.7, 95%CI: 4.8 – 57.6; p<0.001). HDAS was associated with substantially greater odds of experiencing neuropsychiatric, renal and vasculitis disease activity (OR>10, p≤0.001 for all) and of accruing new damage (OR: 2.6, 95% CI: 1.4 – 4.7; p=0.002) during the observation period.

Conclusion:  Patients who ever experience HDAS represent a distinct clinical cohort with worse longitudinal disease outcomes. The occurrence of HDAS can serve as a prognostic indicator applicable to a heterogeneous lupus population.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-high-disease-activity-state-is-an-adverse-prognostic-indicator-in-sle-and-defines-a-clinically-distinct-population/