Abstract：
To describe the clinical and pathological features of biopsy-proven cutaneous vasculitis (CV) associated with SLE, focusing on diagnosis classification and impact on overall SLE activity.
Retrospective multicentric cohort study including SLE patients with biopsy-proven CV identified by (i) data from pathology departments of three university hospitals and (ii) a national call for cases. SLE was defined according to 1997 revised ACR and/or 2019 ACR/EULAR criteria. CV diagnosis was confirmed histologically and classified by using the dermatological addendum of the Chapel Hill classification. SLE activity and flare severity at the time of CV diagnosis were assessed independently of vasculitis items with the SELENA-SLEDAI and SELENA-SLEDAI Flare Index.
Overall, 39 patients were included; 35 (90%) were female. Cutaneous manifestations included mostly palpable purpura (n = 21; 54%) and urticarial lesions (n = 18; 46%); lower limbs were the most common location (n = 33; 85%). Eleven (28%) patients exhibited extracutaneous vasculitis. A higher prevalence of Sjögren\'s syndrome (51%) was found compared with SLE patients without CV from the French referral centre group (12%, P < 0.0001) and the Swiss SLE Cohort (11%, P < 0.0001). CV was mostly classified as urticarial vasculitis (n = 14, 36%) and cryoglobulinaemia (n = 13, 33%). Only 2 (5%) patients had no other cause than SLE to explain the CV. Sixty-one percent of patients had inactive SLE.
SLE-related vasculitis seems very rare and other causes of vasculitis should be ruled out before considering this diagnosis. Moreover, in more than half of patients, CV was not associated with another sign of active SLE.
Retrospective multicentric cohort study including SLE patients with biopsy-proven CV identified by (i) data from pathology departments of three university hospitals and (ii) a national call for cases. SLE was defined according to 1997 revised ACR and/or 2019 ACR/EULAR criteria. CV diagnosis was confirmed histologically and classified by using the dermatological addendum of the Chapel Hill classification. SLE activity and flare severity at the time of CV diagnosis were assessed independently of vasculitis items with the SELENA-SLEDAI and SELENA-SLEDAI Flare Index.
Overall, 39 patients were included; 35 (90%) were female. Cutaneous manifestations included mostly palpable purpura (n = 21; 54%) and urticarial lesions (n = 18; 46%); lower limbs were the most common location (n = 33; 85%). Eleven (28%) patients exhibited extracutaneous vasculitis. A higher prevalence of Sjögren\'s syndrome (51%) was found compared with SLE patients without CV from the French referral centre group (12%, P < 0.0001) and the Swiss SLE Cohort (11%, P < 0.0001). CV was mostly classified as urticarial vasculitis (n = 14, 36%) and cryoglobulinaemia (n = 13, 33%). Only 2 (5%) patients had no other cause than SLE to explain the CV. Sixty-one percent of patients had inactive SLE.
SLE-related vasculitis seems very rare and other causes of vasculitis should be ruled out before considering this diagnosis. Moreover, in more than half of patients, CV was not associated with another sign of active SLE.
摘要：
目的：描述与系统性红斑狼疮（SLE）相关的经活检证实的皮肤血管炎（CV）的临床和病理特征，重点是诊断分类和对整体SLE活动的影响。
方法：回顾性多中心队列研究，包括SLE患者，其活检证实的CV通过1）来自三所大学医院病理科的数据和2）全国病例呼吁进行鉴定。SLE根据1997年修订的ACR和/或2019年ACR/EULAR标准定义。通过组织学确认CV诊断，并使用ChapelHill分类的皮肤病学附录进行分类。用SELENA-SLEDAI和SELENA-SLEDAI耀斑指数独立于血管炎项目评估CV诊断时的SLE活动和耀斑严重程度。
结果：总体而言，包括39例患者；35（90％）为女性。皮肤表现主要包括明显的紫癜（n=21；54％）和荨麻疹病变（n=18；46％）；下肢是最常见的位置（n=33；85％）。11例（28％）患者出现皮肤外血管炎。与来自法国转诊中心组的无CV的SLE患者相比，Sjögren综合征的患病率更高（51％）（12％，p<0.0001)和瑞士SLE队列(11%，p<0.0001)。CV主要分为荨麻疹性血管炎（n=14，36％）和冷球蛋白血症（n=13，33％）。只有2例(5%)患者除SLE外没有其他原因来解释CV。61％的患者患有活动性SLE。
结论：SLE相关性血管炎似乎非常罕见，在考虑诊断前，应排除其他原因引起的血管炎。此外，在超过一半的患者中，CV与活动性SLE的另一个体征无关。
方法：回顾性多中心队列研究，包括SLE患者，其活检证实的CV通过1）来自三所大学医院病理科的数据和2）全国病例呼吁进行鉴定。SLE根据1997年修订的ACR和/或2019年ACR/EULAR标准定义。通过组织学确认CV诊断，并使用ChapelHill分类的皮肤病学附录进行分类。用SELENA-SLEDAI和SELENA-SLEDAI耀斑指数独立于血管炎项目评估CV诊断时的SLE活动和耀斑严重程度。
结果：总体而言，包括39例患者；35（90％）为女性。皮肤表现主要包括明显的紫癜（n=21；54％）和荨麻疹病变（n=18；46％）；下肢是最常见的位置（n=33；85％）。11例（28％）患者出现皮肤外血管炎。与来自法国转诊中心组的无CV的SLE患者相比，Sjögren综合征的患病率更高（51％）（12％，p<0.0001)和瑞士SLE队列(11%，p<0.0001)。CV主要分为荨麻疹性血管炎（n=14，36％）和冷球蛋白血症（n=13，33％）。只有2例(5%)患者除SLE外没有其他原因来解释CV。61％的患者患有活动性SLE。
结论：SLE相关性血管炎似乎非常罕见，在考虑诊断前，应排除其他原因引起的血管炎。此外，在超过一半的患者中，CV与活动性SLE的另一个体征无关。
