Bouiller Kévin, Audia Sylvain, Devilliers Hervé, Collet Evelyne, Aubriot Marie Hélène, Leguy-Seguin Vanessa, Berthier Sabine, Bonniaud Philippe, Chavanet Pascal, Besancenot Jean-François, Vabres Pierre, Martin Laurent, Samson Maxime, Bonnotte Bernard
Service de médecine interne et immunologie clinique Service de médecine interne et maladies systémiques Service de dermatologie Laboratoire d'anatomopathologie Service de pneumologie Service de maladies infectieuses, CHU François Mitterrand, Dijon, France.
Medicine (Baltimore). 2016 Jul;95(28):e4238. doi: 10.1097/MD.0000000000004238.
In this study, outcomes of patients with leukocytoclastic vasculitis (LCV) were analyzed focusing on clinical, histopathology and laboratory findings, relapses, and survival.Data from patients with cutaneous vasculitis diagnosed between January 1, 2000, and December 31, 2010, at Dijon University Hospital (France) were retrospectively reviewed. LCV was defined as perivascular neutrophilic infiltrate, endothelial cell nuclear swelling, extravasation of red blood cells, and/or fibrin deposition in vessels. Patients were classified according to the 2012 Chapel Hill Consensus Conference. Relapses were defined as the recurrence of vasculitis symptoms after a period of remission >1 month. Time to relapse and/or death was calculated from the date of diagnosis. Univariate and multivariate (Cox model) analyses were performed.A total of 112 patients (57 males and 55 females), with a mean age of 60 ± 19 (18-98) years, were analyzed. Overall follow-up was 61 ± 38 months. At diagnosis, all patients had skin lesions, purpura being the most common (n = 83). Lesions were associated with systemic involvement in 55 (51%) patients. Only 41 (36.6%) patients received specific treatment: glucocorticoids in 29 of 41 (70.7%) and immunosuppressants in 9 of 41 (22%). Sixty-two patients (55%) had LCV due to underlying causes, 29 (25.9%) had single-organ cutaneous small vessel vasculitis (SoCSVV), and 21 (18.8%) had unclassifiable LCV. Twenty patients of the cohort (18%) experienced relapse, 14 ± 13 (1-40) months after the diagnosis of LCV. None of the 29 patients with SoCSVV relapsed. Independent risk factors for relapse were vascular thrombosis in the biopsy [hazard ratio (HR) = 4.9; P = 0.017], peripheral neuropathy (HR = 9.8; P = 0.001), hepatitis (HR = 3.1; P = 0.004), and positive antineutrophil cytoplasm antibodies (ANCA, HR = 5.9 P = 0.005). In contrast, SoCSVV was a protective factor for relapse (HR = 0.12; P = 0.043).The 1-, 3-, and 6-year overall survival rates were 99%, 83%, and 71%, respectively, with no difference between relapsers and nonrelapsers (P = 0.960) or between SoCSVV and unclassifiable LCV (P = 0.588).This study demonstrates that global survival for LCV patients is good but relapses remain frequent, especially when the cutaneous biopsy shows vascular thrombosis, or in patients with peripheral neuropathy or hepatitis. Conversely, SoCSVV is a protective factor for relapse.
在本研究中，我们对白细胞破碎性血管炎（LCV）患者的临床、组织病理学、实验室检查结果、复发情况及生存率进行了分析。回顾性分析了2000年1月1日至2010年12月31日在法国第戎大学医院诊断为皮肤血管炎患者的数据。LCV的定义为血管周围中性粒细胞浸润、内皮细胞核肿胀、红细胞外渗和/或血管内纤维蛋白沉积。患者根据2012年查珀尔希尔共识会议进行分类。复发定义为缓解超过1个月后血管炎症状复发。从诊断日期开始计算复发和/或死亡时间。进行了单因素和多因素（Cox模型）分析。
共分析了112例患者（57例男性和55例女性），平均年龄为60±19（18 - 98）岁。总体随访时间为61±38个月。诊断时，所有患者均有皮肤病变，紫癜最为常见（n = 83）。55例（51%）患者的病变与全身受累有关。仅41例（36.6%）患者接受了特异性治疗：41例中的29例（70.7%）使用了糖皮质激素，41例中的9例（22%）使用了免疫抑制剂。62例（55%）患者的LCV有潜在病因，29例（25.9%）患有单器官皮肤小血管血管炎（SoCSVV），21例（18.8%）患有无法分类的LCV。该队列中的20例患者（18%）出现复发，在LCV诊断后14±13（1 - 40）个月。29例SoCSVV患者均未复发。复发的独立危险因素为活检中有血管血栓形成[风险比（HR）= 4.9；P = 0.017]、周围神经病变（HR = 9.8；P = 0.001）、肝炎（HR = 3.1；P = 0.004）以及抗中性粒细胞胞浆抗体（ANCA）阳性（HR = 5.9；P = 0.005）。相反，SoCSVV是复发的保护因素（HR = 0.12；P = 0.043）。
1年、3年和6年的总生存率分别为99%、83%和71%，复发患者和未复发患者之间（P = 0.960）或SoCSVV和无法分类的LCV之间（P =
