Abstract

Objectives: We sought to examine the disease course of High Disease Activity Status (HDAS) patients and their different disease patterns in a real-world longitudinal cohort. Methods: All consenting patients of the Monash Lupus Cohort who had at least 12 months of observation were included. HDAS was defined as SLEDAI-2K³10 ever, and HDAS episode as the period from the first HDAS clinic visit until attainment of Lupus Low Disease Activity State (LLDAS). We examined the associations of different HDAS patterns with the likelihood of damage accrual. Results: Of 342 SLE patients, 151 experienced HDAS at least once, accounting for 298 HDAS episodes. The majority of HDAS patients (76∙2%) experienced Recurrent HDAS (>1 HDAS visit), and a smaller subset (47∙7%) had Persistent HDAS (consecutive HDAS visits for longer than 2 months. Recurrent or Persistent HDAS patients were younger at diagnosis and more likely to experience renal or serositis manifestations; persistent HDAS patients were also more likely to experience neurological manifestations. Baseline SLEDAI greater than 10 was associated with longer HDAS episodes. Recurrent or Persistent HDAS were both associated with an increased likelihood of damage accrual. Total duration of HDAS episode greater than 2 years and experiencing multiple HDAS episodes ( > 4) were also associated with an increased likelihood of damage accrual (OR 1∙80, 95%CI 1∙08 - 2∙97, p=0∙02, and OR 3∙31, 95% CI 1∙66-13∙26, p=0∙01 respectively). Conclusion: HDAS episodes have a highly variable course. Recurrent and Persistent HDAS, and longer duration of HDAS episodes, increased risk of damage accrual. HDAS is a major signifier of severity in SLE. Funding Statement: Funding was received for the conduct of the Australia Lupus Registry and Biobank at the Monash Lupus Clinic, in terms of sponsorship and unrestricted educational grants (AH, RK, JB, HN, EM) from Merck KGaA, GlaxoSmithKline, UCB, and Astra Zeneca. Merck KGaA (YS, AK, OG) provided specific financial support for this study. Declaration of Interests: All authors have no relevant conflicts of interest in terms of financial and personal relationships. Ethics Approval Statement: The study was approved by the Monash Health Human Research Ethics Committee.

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