Rheumatology and Clinical Immunology Unit, Attikon University Hospital, Athens, Greece.
Department of Rheumatology and Clinical Immunology, University Hospital of Heraklion, Heraklion, Greece.
Lupus. 2024 Oct;33(11):1248-1253. doi: 10.1177/09612033241273023. Epub 2024 Aug 4.
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.
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.
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% ( = 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.
Anifrolumab is highly effective in various skin manifestations of SLE, even after prior failure to multiple treatments.
皮肤受累是红斑狼疮（SLE）的常见表现，但可能对常规治疗产生抵抗。我们旨在评估 anifrolumab（ANI）在治疗难治性系统性红斑狼疮皮肤表现方面的疗效。
来自希腊三家风湿病科的难治性皮肤性红斑狼疮患者的病例系列。疗效评估指标为系统性红斑狼疮疾病活动度指数 2000（SLEDAI-2K）、医生总体评估（PGA）和皮肤狼疮疾病面积和严重程度指数（CLASI）的改善情况。皮肤临床相关改善定义为自基线值下降≥50%（CLASI50）。
18 名患者接受了 ANI 治疗；所有患者在基线时均有活动期皮肤受累。ANI 起始时 SLEDAI 和 PGA 的平均值分别为 7.4（2.7）和 1.4（0.5），平均泼尼松剂量为 4.9（4.5）mg/天。基线时 CLASI（活动/损害）的平均值为 13.9（9.7）/2.9（4.6）。在开始接受 ANI 治疗之前，患者平均已接受 6.3（1.5）种免疫调节剂（包括羟氯喹和糖皮质激素）治疗，但疗效不佳。平均随访 8.5（4.6）个月后，89%（=16/18）的患者总体狼疮和皮肤疾病活动度以及糖皮质激素减药方面均有显著改善。末次就诊时的 SLEDAI 和 CLASI 平均值分别为 3.4（1.9）和 2.1（2.4）/1.4（2.2），平均泼尼松日剂量降至 2.4（2.2）mg。值得注意的是，在这组难治性患者中，16/18（89%）的患者达到了 CLASI50。1 例患者因水痘带状疱疹病毒感染在接受 4 次输注后停用 ANI，1 例患者在接受 ANI 治疗后最初有反应，但因 COVID-19 感染而暂时停药，导致皮肤病情再次发作。两名（11.1%）和 11 名（61.1%）患者分别达到了 DORIS 缓解和 LLDAS。
即使在先前对多种治疗方法耐药后，ANI 对各种皮肤性 SLE 表现均具有高度疗效。
