LLDAS is an attainable treat-to-target goal in childhood-onset SLE

Abstract

ObjectivesTo study whether clinical remission (CR) and Low Lupus Disease Activity State (LLDAS) are achievable goals in childhood-onset SLE.MethodsData on medication use and disease activity were prospectively collected. LLDAS was defined as Safety of Estrogen in Lupus Erythematosus National Assesment-SLE disease Activity Index (SELENA-SLEDAI) ≤4 with zero scores for renal, Central Nervous System (CNS), serositis, vasculitis and constitutional components, no increase in any SLEDAI component since the previous visit, PGA ≤1, and prednisone dose ≤7.5 mg/day. CR on treatment (Tx) was defined as a Physician Global Assessment <0.5, SELENA-SLEDAI=0, with prednisone ≤5 mg/day and maintenance treatment with immunosuppressives. CR off Tx was the same but without prednisone or other immunosuppressive usage.Results51 patients (700 visits) were included. Within 3 months after diagnosis, 94.1% of children were treated with hydroxychloroquine and 60.8% with prednisone. Prednisone dosage decreased from a median of 0.74 mg/kg/day at diagnosis to 0.44 mg/kg/day at 3 months and 0.16 mg/kg/day at 6 months after diagnosis. Use of mycophenolate mofetil increased from 25.5% to 56.9% within 6 months after diagnosis. All children achieved LLDAS (median 186 days) and 72.5% remained in LLDAS >50% of time. 52.9% children achieved CR on Tx, and only 21.6% children achieved CR off Tx.ConclusionsLLDAS is an attainable treat-to-target goal in contrast to CR on and off Tx. Even more, LLDAS can be reached with limited use of corticosteroids with early introduction of immunosuppressives.

Efficacy and safety of belimumab for the treatment of refractory childhood-onset systemic lupus erythematosus: A single-center, real-world, retrospective study

“…LLDAS is a reachable goal of T2T for the treatment of cSLE. For example, in Wahadat’s study, the follow-up of 51 cSLE patients showed that all patients achieved LLDAS after 186 days of treatment, on average ( 26 ). Another study has demonstrated that belimumab reduced the disease activity as evaluated by the SLEDAI-2K score and the risk of recurrence ( 27 ).…”

Towards development of treat to target (T2T) in childhood-onset systemic lupus erythematosus: PReS-endorsed overarching principles and points-to-consider from an international task force

“…17 Similarly, a Dutch cSLE Cohort (n=51, 700 visits, median follow-up 3 years), demonstrated CR on/off-treatment to be achieved by 53%/22% of patients, respectively. 18 In UK patients, achievement of CR on-treatment was associated with significantly reduced hazards of severe flare (HRs 0.19, 95% CI 0.15 to 0.24) and new damage (HR 0.27, 95% CI 0.14,0.50, both p<0.001). Achievement of CR off-treatment was associated with further reduction in the hazards of severe flare (HR 0.13 (95% CI 0.09 to 0.20), 95% CI 0.15,0.24) and new damage (HR 0.10, 95% CI 0.07 to 0.16, both p<0.001).…”

“…T2T is part of routine clinical care in rheumatoid arthritis, hypertension and diabetes, underpinned by studies demonstrating improvements in long-term outcomes. [12][13][14][15] There is considerable international interest in T2T in cSLE, [16][17][18] and aSLE, [19][20][21] with the hope that T2T will enable use of existing treatments in a structured way, with the aim of controlling disease activity, preventing organ damage, improving HRQOL, and ultimately improving survival. [21][22][23] Internationally agreed principles and recommendations for T2T have laid the foundation for T2T in aSLE, 21 and Juvenile Idiopathic Arthritis (JIA).…”

Current views on lupus in children

“…A single centre study from the Netherlands demonstrated that all jSLE patients reached LLDAS within a median of 6 months, remaining in LLDAS greater than 50% of follow-up time. Factors independently predicting LLDAS attainment by 3 months included prednisolone dosage at 3 months, and treatment with MMF [135 ▪ ]. The benefit of early MMF treatment is also highlighted by a recent study assessing glucocorticoid discontinuation in jSLE.…”