Bobek Dubravka, Vuković Jurica, Malenica Branko, Bojanić Katarina, Rukavina Iva, Jelušić Marija
Assist Prof. Marija Jelušić, MD, PhD, Department of Pediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Kišpatićeva 12, 10000 Zagreb, Croatia;
Acta Dermatovenerol Croat. 2014;22(4):264-70.
Juvenile systemic lupus erythematosus (JSLE) is a systemic autoimmune chronic disease that can affect any part of the body. It is characterized by the formation of antibodies against nuclear antigens. Vasculitis may be found in SLE, but it scarcely complies with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) criteria. We report five cases of severe JSLE associated with AAV diagnosed between 1991 and 2013 in three university-based tertiary care centers. The patients (3 girls and 2 boys, aged 12 to 17) presented with a severe clinical picture and the following features: cytopenia (n=5), autoimmune hepatitis (n=3), lupus nephritis (n=1), pancreatitis (n=1), secondary antiphospholipid syndrome (n=2), impending respiratory failure (n=2), and gastrointestinal bleeding (n=1). All patients were proteinase 3 (PR3) ANCA positive, while two of them were myeloperoxidase (MPO) and PR3 ANCAs positive at the same time. They were treated with corticosteroids and immunosuppressive drugs. Remission of the disease was achieved in three patients. The course of the disease was worsening in two patients and we included rituximab (anti-CD20) in therapy. All of our patients presented as the most severe SLE patients, who must be diagnosed as soon as possible and treated very intensively. Since the comorbidity of JSLE and AAV occurs very rarely in children, presentation of such patients, their clinical pictures, treatment, and the course of the diseases are experiences that can be of great help.
青少年系统性红斑狼疮（JSLE）是一种全身性自身免疫性慢性病，可累及身体的任何部位。其特征是形成针对核抗原的抗体。血管炎可见于系统性红斑狼疮（SLE），但很少符合抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）的标准。我们报告了1991年至2013年间在三个大学附属三级医疗中心诊断出的5例与AAV相关的重症JSLE病例。这些患者（3名女孩和2名男孩，年龄在12至17岁之间）临床表现严重，具有以下特征：血细胞减少（n = 5）、自身免疫性肝炎（n = 3）、狼疮性肾炎（n = 1）、胰腺炎（n = 1）、继发性抗磷脂综合征（n = 2）、即将发生的呼吸衰竭（n = 2）和胃肠道出血（n = 1）。所有患者蛋白酶3（PR3）-ANCA均为阳性，其中2例同时为髓过氧化物酶（MPO）和PR3-ANCA阳性。他们接受了糖皮质激素和免疫抑制药物治疗。3例患者病情缓解。2例患者病情恶化，我们在治疗中加入了利妥昔单抗（抗CD20）。我们所有的患者均表现为最严重的SLE患者，必须尽快诊断并进行强化治疗。由于JSLE和AAV的合并症在儿童中非常罕见，这些患者的临床表现、治疗及疾病进程都是非常有帮助的经验。
