全身型幼年型特发性关节炎31例临床分析

Clinical Analysis of 31 Cases of Systemic Juvenile Idiopathic Arthritis

摘要: 目的：分析全身型幼年型特发性关节炎(SJIA)的临床表现、辅助检查结果、治疗措施、临床转归，为临床诊断和治疗SJIA提供依据。方法：通过青岛大学附属医院信息管理系统及医疗科研大数据平台收集2015年5月到2022年9月住院治疗的31例SJIA患者的资料，包括一般情况、临床表现、辅助检查结果、治疗措施、预后等。结果：31例SJIA，男19人(61.29%)，女12人(38.71%)，男女比例约1.5:1，平均年龄为(6.25 ± 3.39)。临床表现无特异性，均有发热，热峰大部分波动于39℃，最低38℃，最高41℃，呈弛张热；18人(58%)伴有皮疹，热起疹出，热退疹退，3例为全身性，余15例散发于身体各部位，20例(64.5%)有关节痛，8例(25.8%)有关节肿，大小关节均可受累。白细胞总数(WBC) ≥ 15 × 10⁹/L 21例(67.74%)，轻中度贫血10例(32.27%)，血小板升高19例(61.29%)，C反应蛋白(CRP)检测30例，血清铁蛋白检测26例，均升高，红细胞沉降率(ESR)检测27例，26例升高(96.3%)，24例检测淋巴细胞亚群，其中CD3升高11例(45.83%)，CD4降低6例(25%)，CD4升高9例(37.5%)，CD8升高8例(33.33%)，CD19升高8例(33.33%)，NK细胞降低21例(87.5%)，2O例检测细胞因子，其中IL-6升高17例(85%)，19例检测免疫球蛋白，6例正常，13例结果异常(68.42%)，其中11例IgE增高，4例IgG升高，2例IgA升高，1例IgA降低，1例IgM升高。经查体及影像学检查，19例有淋巴结肿大，4例肝大，5例脾大，8例浆膜腔积液，关节腔积液13例，骨髓水肿5例，关节周围组织肿胀3例，滑膜炎2例，滑膜囊肿1例，10例胸部CT异常。经激素、非甾体抗炎药、改变病情抗风湿药(DMARDs)等治疗，部分患儿因病情反复，需生物制剂治疗，10例并发巨噬细胞活化综合征(macrophage activation syndrome, MAS)，经个体化治疗后均缓解。结论：SJIA临床表现无特异性，可有持续高热、皮疹、关节疼痛、肿胀等，可伴肝、脾、淋巴结等的肿大，CRP、ESR可明显升高，部分有细胞、体液免疫的异常。如持续高热、铁蛋白持续升高、肝功能异常等，警惕MAS的存在，早期诊断，及早予激素冲击、丙球、环孢素A、生物制剂等的治疗，是改善预后的关键。

Abstract: Objective: To analyze the clinical manifestations, auxiliary examination results, treatment measures and clinical outcomes of systemic juvenile idiopathic arthritis (SJIA), and to provide evidence for clinical diagnosis and treatment of SJIA. Methods: The data of 31 patients with SJIA hospitalized in the Affiliated Hospital of Qingdao University from May 2015 to September 2022 were collected through the information management system and medical research big data platform, including general information, clinical manifestations, auxiliary examination results, treatment measures, prognosis, etc. Results: There were 19 males (61.29%) and 12 females (38.71%), with a male to female ratio of about 1.5:1. The mean age was (6.25 ± 3.39). The clinical manifestations were nonspecific. All patients had fever, and most of the peak temperature fluctuated at 39˚C, with a minimum of 38˚C and a maximum of 41˚C. Eighteen patients (58%) had skin rashes, which were generalized in 3 patients and sporadic in 15 patients. Twenty patients (64.5%) had joint pain and 8 patients (25.8%) had joint swelling. There were 21 cases (67.74%) with white blood cell count (WBC) ≥ 15 × 10⁹/L, 10 cases (32.27%) with mild to moderate anemia, 19 cases (61.29%) with thrombocytopenia, 30 cases with C-reactive protein (CRP) detection, 26 cases with serum ferritin detection, all of which were elevated. Erythrocyte sedimentation rate (ESR) was detected in 27 cases, and 26 cases (96.3%) were increased. Lymphocyte subsets were detected in 24 cases, including CD3 increased in 11 cases (45.83%), CD4 decreased in 6 cases (25%), CD4 increased in 9 cases (37.5%), CD8 increased in 8 cases (33.33%), CD19 increased in 8 cases (33.33%). NK cells decreased in 21 cases (87.5%), cytokines were detected in 20 cases, of which IL-6 was increased in 17 cases (85%), immunoglobulin was detected in 19 cases, 6 cases were normal, 13 cases were abnormal (68.42%), including 11 cases of IgE increased, 4 cases of IgG increased, 2 cases of IgA increased, 1 case of IgA decreased, and 1 case of IgM increased. Physical examination and imaging examination showed lymphadenopathy in 19 cases, hepatomegaly in 4 cases, splenomegaly in 5 cases, serous effusion in 8 cases, joint effusion in 13 cases, bone marrow edema in 5 cases, periarticular tissue swelling in 3 cases, synovitis in 2 cases, synovial cyst in 1 case, and abnormal chest CT in 10 cases. After treatment with hormones, non-steroidal anti-inflammatory drugs, disease-modifying antirheumatic drugs (DMARDs), some children needed biological agents due to the recurrence of the disease, 10 cases were complicated with macrophage activation syndrome (MAS), and all were relieved after individualized treatment. Conclusions: The clinical manifestations of SJIA are nonspecific, such as persistent high fever, rash, joint pain, swelling, etc., accompanied by enlargement of liver, spleen and lymph nodes, etc. CRP and ESR may be significantly increased, and some may have abnormalities of cellular and humoral immunity. The key to improve the prognosis is to be alert to the presence of MAS, such as persistent high fever, persistently elevated ferritin, abnormal liver function, early diagnosis, and early treatment with hormone pulse, globulin, cyclosporine A, biological agents, etc.