Background/Purpose: High Disease Activity Status (HDAS), defined as ever attainment of SLEDAI-2k of 10 or greater, may be a useful indicator of overall disease severity in systemic lupus erythematosus. The natural history and disease course following episodes of HDAS has not been previously described.

Methods: Using prospectively collected data from the Australian Lupus Registry and Biobank, we examined the patterns and consequences of HDAS. A HDAS episode (until resolution) was defined as the interval from first visit with a recorded SLEDAI -2K≥10, to the time when patient achieved Low Lupus Disease Activity State (LLDAS)¹.  Recurrent HDAS was defined as patients who had at least 2 visits with SLEDAI-2k≥10 that were at least 2 months apart. Persistent HDAS was defined as a subset of recurrent HDAS patients in whom SLEDAI-2k was ≥10 at consecutive visits. The associations of these time course patterns with clinical outcomes were analysed.

Results: Data on 286 patients followed for 4.5 +/- 3.3 years were studied. A total of 128 patients had at least one visit that met the criteria for HDAS, accounting for 254 HDAS episodes. The duration of HDAS episodes was variable (median 133 days, IQR 70-245 days), and were significantly longer in patients entering an HDAS episode with SLEDAI-2K > 10 compared to those entering the HDAS episode with SLEDAI-2K = 10 (p=0.003, unpaired heteroscedactic t-test). Accordingly, the likelihood of an HDAS episode lasting > 12 months was 1.89 (95% CI 1.03-3.46, p=0.04) if starting SLEDAI was >10 compared to =10. The number of HDAS episodes increased the odds of damage accrual, with an odds ratio for increased SLICC damage of 4.12 (p< 0.001) if the patient experienced 2 episodes during the observed period (Table 1).

Most HDAS patients had recurrent HDAS (95/128, 74%). Forty-four patients fulfilled the definition of persistent HDAS. Compared to patients who never experienced HDAS, the risk of damage accrual was markedly increased in recurrent HDAS patients, with an OR 3.1 (95% CI 1.82-5.32, p< 0.001). Persistent HDAS patients have the highest risk of damage accrual with an OR 7.13 (95% CI 3.45-15.62, p< 0.001).

Conclusion: HDAS is a clinically relevant SLE disease severity measure, but its disease course can be highly variable. The risk of damage accrual increased progressively according to exposure to HDAS. Patients with persistent HDAS are at the highest risk of damage accrual.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/patterns-of-high-disease-activity-status-and-outcomes-in-systemic-lupus-erythematosus/