JAAD-D-21-03349-SupplementalMaterial

SUPPLEMENTAL MATERIAL Supplemental Figure 1. Flowchart Flowchart reporting the clinical profile of DM patients included in the study and the number of patients analyzed at last follow-up. Supplemental Figure 2. Cutaneous improvement to JAK inhibitor in a patient at follow-up Illustrative case (patient 4) with skin refractory dermatomyositis demonstrating a cutaneous response to ruxolitinib. CDASI at baseline was 49 and significantly decreased to 30 at 3-month. Clinical improvement was sustained (CDASI of 26) at 6-month follow-up. Prednisone was decreased from 10 to 7 mg/day over this period of time. Supplemental Figure 3. Manual muscle testing evolution following JAK inhibitor treatment Mean±SD manual MMT8 of 120±31, 121±29, 128±26 and 132±124 at baseline (M0), 3-month, 6-month and last follow-up, respectively. M0: baseline; M3: 3-month; M6: 6-month; FU: follow-up; MMT: manual muscle testing. Data shown as mean±SD Supplemental Figure 4. Treatment burden evolution following JAK inhibitor treatment Percentage of patients remaining with IVIg and/or prednisone 5mg/day at follow-up. IVIg was stopped in 1 patient at 6-month and in 4 patients at last follow-up. Prednisone decrease to ≤5 mg/day was achieved in 1, 2 and 10 patients at 3-month, 6-month and last follow-up, respectively. IVIg: intravenous immunoglobulin; Pred: prednisone; M0: baseline; M3: 3-month; M6: 6-month; FU: follow-up. Data shown as percentage Supplemental Figure 5. IFN alpha at 3 months Non-significant trend of IFN alpha decrease from mean±SD 1612±4316 fg/mL to 1352±3722 fg/mL between baseline and 3-month. IFN: interferon补充材料 补充图1：流程图 展示本研究纳入的皮肌炎（Dermatomyositis, DM）患者的临床特征，以及末次随访时纳入分析的患者例数。 补充图2：JAK抑制剂（Janus Kinase inhibitor）治疗后患者皮肤改善情况 本示例病例为患者4，其患有难治性皮肤型皮肌炎，对芦可替尼（ruxolitinib）产生皮肤应答。基线时CDASI（Cutaneous Dermatomyositis Disease Area and Severity Index）评分为49，随访3个月时显著降至30；6个月随访时临床症状持续改善，CDASI评分为26。此治疗周期内，泼尼松（prednisone）用量从10mg/日减至7mg/日。 补充图3：JAK抑制剂治疗后徒手肌力测试的变化 基线（M0）、3个月、6个月及末次随访时的8项徒手肌力测试（manual muscle testing, MMT）评分（MMT8）均值±标准差分别为120±31、121±29、128±26及132±124。其中M0代表基线，M3代表3个月，M6代表6个月，FU代表随访，MMT代表徒手肌力检查。数据以均值±标准差形式展示。 补充图4：JAK抑制剂治疗后治疗负担的变化 随访时仍接受静脉注射免疫球蛋白（intravenous immunoglobulin, IVIg）和/或泼尼松（prednisone）≤5mg/日的患者占比。6个月时1例患者停用IVIg，末次随访时4例患者停用IVIg。在3个月、6个月及末次随访时，分别有1例、2例及10例患者将泼尼松用量降至≤5mg/日。数据以百分比形式展示。 补充图5：3个月随访时的干扰素α水平 基线至3个月时，干扰素（interferon, IFN）α水平从均值±标准差1612±4316 fg/mL降至1352±3722 fg/mL，未呈现显著下降趋势。