阿尼单抗治疗系统性红斑狼疮伴多发性难治性皮肤病:18例患者的病例系列。

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2024-10-01 Epub Date: 2024-08-04 DOI:10.1177/09612033241273023
Sofia Flouda, Evgenia Emmanouilidou, Anastasios Karamanakos, Dimitra Koumaki, Dimitrios Katsifis-Nezis, Argyro Repa, George Bertsias, Dimitrios Boumpas, Antonis Fanouriakis
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引用次数: 0

摘要

目的:皮肤受累是系统性红斑狼疮(SLE)的常见症状,但可能对常规治疗产生耐药性。我们试图评估阿尼洛单抗(ANI)对难治性皮肤表现的疗效:方法:希腊三个风湿病科的难治性皮肤系统性红斑狼疮患者病例系列。结果测量指标为系统性红斑狼疮疾病活动指数2000(SLEDAI-2K)、医生总体评估(PGA)和皮肤红斑狼疮疾病面积和严重程度指数(CLASI)的改善情况。与临床相关的皮肤改善定义为比基线值下降≥50%(CLASI50):18名患者接受了ANI治疗;所有患者基线时均有活动性皮肤受累。开始 ANI 时的 SLEDAI 和 PGA 平均值(标清)分别为 7.4 (2.7) 和 1.4 (0.5),泼尼松平均剂量为 4.9 (4.5) 毫克/天。基线时的平均 CLASI(活动/损伤)为 13.9 (9.7)/2.9 (4.6)。在开始使用 ANI 之前,患者对平均 6.3(1.5)种免疫调节药物(包括羟氯喹和糖皮质激素)呈难治性。在平均8.5(4.6)个月后,89%的患者(n = 16/18)的一般狼疮和皮肤疾病活动明显改善,糖皮质激素也逐渐减少。最后一次就诊时的平均 SLEDAI 和平均 CLASI 分别为 3.4 (1.9) 和 2.1 (2.4)/1.4 (2.2),平均每日泼尼松剂量降至 2.4 (2.2)。值得注意的是,在这组高度难治性患者中,有16/18(89%)名患者达到了CLASI50。一名患者因水痘-带状疱疹病毒感染在输注 4 次后停止 ANI,一名最初对 ANI 治疗有反应的患者因感染 Covid 19 而暂时停止治疗,导致皮肤复发。分别有2名(11.1%)和11名(61.1%)患者获得了DORIS缓解和LLDAS:结论:安非罗单抗对系统性红斑狼疮的各种皮肤表现非常有效,即使是在多种治疗失败之后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anifrolumab for systemic lupus erythematosus with multi-refractory skin disease: A case series of 18 patients.

Objective: Skin involvement is common in systemic lupus erythematosus (SLE), but may be resistant to conventional treatment. We sought to evaluate the efficacy of anifrolumab (ANI) in refractory cutaneous manifestations of SLE.

Methods: Case series of patients with refractory cutaneous SLE from three Rheumatology Departments in Greece. Outcome measures were improvement in Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), physician global assessment (PGA) and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Clinically relevant improvement in skin was defined as decrease ≥50% (CLASI50) from baseline values.

Results: Eighteen patients received ANI; all had active skin involvement at baseline. Mean (SD) SLEDAI and PGA at ANI initiation were 7.4 (2.7) and 1.4 (0.5), respectively, with a mean prednisone dose 4.9 (4.5) mg/day. Mean CLASI (Activity/Damage) at baseline was 13.9 (9.7)/2.9 (4.6). Patients were refractory to a mean 6.3 (1.5) immunomodulatory agents (including hydroxychloroquine and glucocorticoids) before the initiation of ANI. After a mean 8.5 (4.6) months, 89% (n = 16/18) of patients demonstrated significant improvement in general lupus and cutaneous disease activity, and glucocorticoid tapering. Mean SLEDAI and mean CLASI at last visit were 3.4 (1.9) and 2.1 (2.4)/1.4 (2.2), respectively, and mean daily prednisone dose decreased to 2.4 (2.2). Of note, in this group of highly refractory patients CLASI50 was achieved in 16/18 (89%) patients. One patient discontinued ANI after 4 infusions due to a varicella-zoster virus infection and one patient, who initially responded to treatment with ANI, experienced a skin flare due to temporary discontinuation due to Covid 19 infection. DORIS remission and LLDAS were attained in two (11.1%) and eleven (61.1%) patients, respectively.

Conclusion: Anifrolumab is highly effective in various skin manifestations of SLE, even after prior failure to multiple treatments.

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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
期刊介绍: The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…
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