Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands.
Department of Paediatric Rheumatology, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands.
Lupus Sci Med. 2021 Dec;8(1). doi: 10.1136/lupus-2021-000571.
To study whether clinical remission (CR) and Low Lupus Disease Activity State (LLDAS) are achievable goals in childhood-onset SLE.
Data 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.
51 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.
LLDAS 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.
研究儿童发病的系统性红斑狼疮(SLE)能否实现临床缓解(CR)和低疾病活动状态(LLDAS)。
前瞻性收集药物使用和疾病活动数据。LLDAS 定义为安全性评估-红斑狼疮疾病活动指数(SELENA-SLEDAI)≤4,无肾脏、中枢神经系统(CNS)、浆膜炎、血管炎和全身表现,与前次就诊相比 SLEDAI 评分无任何增加,医师总体评估(PGA)≤1,泼尼松剂量≤7.5mg/天。治疗时 CR 定义为医师总体评估<0.5,SELENA-SLEDAI=0,泼尼松≤5mg/天,且维持免疫抑制剂治疗。停止治疗时 CR 定义与治疗时 CR 相同,但无泼尼松或其他免疫抑制剂的使用。
共纳入 51 例(700 次就诊)患儿。确诊后 3 个月内,94.1%患儿接受羟氯喹治疗,60.8%患儿接受泼尼松治疗。泼尼松剂量从诊断时的中位数 0.74mg/kg/天降至 3 个月时的 0.44mg/kg/天,6 个月时降至 0.16mg/kg/天。诊断后 6 个月内,霉酚酸酯的使用率从 25.5%增加至 56.9%。所有患儿均达到 LLDAS(中位数 186 天),50%以上时间内患儿处于 LLDAS>50%。52.9%患儿达到治疗时 CR,仅 21.6%患儿达到停止治疗时 CR。
与停止治疗时 CR 相比,LLDAS 是可实现的治疗目标。此外,通过早期使用免疫抑制剂,限制糖皮质激素的使用,也可以达到 LLDAS。