Remission and low disease activity state in childhood-onset systemic lupus erythematosus: differential impact on damage accrual in a Latin American cohort (GLADEL)

Objective To examine the impact of different disease activity states (DAS) on outcomes in a longitudinal inception childhood-onset SLE cohort. Materials and methods Three DAS were included—remission: clinical SLE DAS (SLE Disease Activity Index (SLEDAI)=0, prednisone (≤5 mg/day) and/or immunosuppressants (IS) (maintenance dose)); lupus low DAS (LLDAS): SLEDAI ≤4 with 0 scores for major organ involvement, no increase in any SLEDAI component since the previous visit, on prednisone (≤7.5 mg/day) and/or IS (maintenance dose); and active disease otherwise. The association of these DAS with new damage (increase of at least 1 point in the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI)), severe new damage (an increase of ≥3 points in the SDI) and mortality (any cause) at any time during the follow-up was examined using Cox proportional hazards regression models. Results The majority of the 212 patients included had active disease at baseline (84%). There was no association between LLDAS and lower risk of new damage or severe new damage (HR 0.98, 95% CI 0.43 to 2.22, p=0.955 and HR 0.40, 95% CI 0.04 to 3.62, p=0.415, respectively). Remission was associated with lower risk of new damage (HR 0.36, 95% CI 0.15 to 0.91, p=0.030), but it had no impact on mortality (HR 0.45, 95% CI 0.06 to 3.66, p=0.454). Conclusions Attaining LLDAS and remission was lower in our cohort than in other contemporary paediatric lupus cohorts. Remission but not LLDAS was associated with lower risk of new damage over 4.3 years of follow-up. None of these DAS were associated with mortality.