Trindade Vitor C, Bonfá Eloisa, Sakamoto Ana P, Terreri Maria T, Aikawa Nádia E, Fiorot Fernanda J, Pitta Ana C, Balbi Verena A, N Rabelo Carlos, Silva Marco F, Islabão Aline G, Novak Glaucia V, Kozu Katia T, Buscatti Izabel M, Campos Lucia M, Sallum Adriana Me, Assad Ana P, Magalhães Claudia S, Marini Roberto, Fonseca Adriana R, Sztajnbok Flavio R, Santos Maria C, Bica Blanca E, Sena Evaldo G, Moraes Ana J, Robazzi Teresa C, Spelling Paulo F, Scheibel Iloite M, Cavalcanti Andre S, Matos Erica N, Guimarães Luciano J, Santos Flavia P, Carvalho Luciana M, Carneiro-Sampaio Magda, Ferraro Alexandre A, Silva Clovis A
Pediatric Rheumatology Unit, Instituto da Criança e do Adolescente, Hospital das Clínicas HCFMUSP, Sao Paulo, Brazil.
Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
Lupus. 2025 Mar;34(3):292-299. doi: 10.1177/09612033251317357. Epub 2025 Jan 27.
To identify clusters of autoantibodies in a large cSLE population and to verify possible associations between different autoantibody clusters and the following variables: demographic data, cumulative clinical and laboratory manifestations, disease activity, cumulative damage and mortality. A cross-sectional study was performed in 27 Pediatric Rheumatology University centers, including 912 cSLE patients. The frequencies of seven selected autoantibodies (anti-dsDNA, anti-Ro/SSA, anti-La/SSB, anti-Sm, anti-RNP, aCL IgM and/or IgG and LA) were used for cluster analysis using the K-means method. Four distinctive antibody clusters were identified. Cluster 1 ( = 322; 35.31%) was characterized by anti-dsDNA (61.18%), low frequency of antiphospholipid antibodies (<10%), and lower frequency of cutaneous, articular manifestation ( < 0.05) and hypocomplementemia ( < 0.001) compared to the other groups. Cluster 2 ( = 158; 17.32%) comprised anti-dsDNA (93.04%), aCL (87.34%) and LA (39.87%) and higher frequencies of thrombocytopenia ( = 0.006) and antiphospholipid syndrome ( < 0.001) than the other clusters. Cluster 3 ( = 177; 19.41%) was characterized by anti-dsDNA (81.36%), anti-Sm (100%) and anti-RNP (44.63%) antibodies, as well as a higher frequency of proteinuria compared to cluster 4 (58.15% vs 56.13% vs 64.00% vs 49.80%, = 0.031). Cluster 4 ( = 255; 27.96%) consisted of all 7 autoantibodies, with predominance of anti-dsDNA (72.55%), anti-Ro/SSA (89.8%) and anti-La/SSB (45.88%), with no specific clinical pattern, except by higher pulmonary damage ( = 0.017). Our study suggests that, within the context of cSLE, the coexistence of anti-dsDNA with antiphospholipid autoantibodies is linked to an elevated incidence of antiphospholipid syndrome. This association does not coincide with a proportionate increase in the occurrence of nephritis. Conversely, the cluster of anti-dsDNA with anti-Ro/SSA and anti-La/SSB antibodies was associated with pulmonary damage, requiring close surveillance.
为了在一大群儿童系统性红斑狼疮（cSLE）患者中识别自身抗体簇，并验证不同自身抗体簇与以下变量之间可能存在的关联：人口统计学数据、累积临床和实验室表现、疾病活动度、累积损伤和死亡率。在27个儿科风湿病大学中心进行了一项横断面研究，纳入了912例cSLE患者。使用K均值法对七种选定自身抗体（抗双链DNA、抗Ro/SSA、抗La/SSB、抗Sm、抗RNP、抗心磷脂IgM和/或IgG以及狼疮抗凝物）的频率进行聚类分析。识别出四个不同的抗体簇。簇1（n = 322；35.31%）的特征是抗双链DNA（61.18%）、抗磷脂抗体频率低（<10%），与其他组相比，皮肤、关节表现（<0.05）和低补体血症（<0.001）的频率较低。簇2（n = 158；17.32%）包括抗双链DNA（93.04%）、抗心磷脂（87.34%）和狼疮抗凝物（39.87%），血小板减少症（P = 0.006）和抗磷脂综合征（<0.001）的频率高于其他簇。簇3（n = 177；19.41%）的特征是抗双链DNA（81.36%）、抗Sm（100%）和抗RNP（44.63%）抗体，与簇4相比蛋白尿频率更高（58.15%对56.13%对64.00%对49.80%，P = 0.031）。簇4（n = 255；27.96%）由所有7种自身抗体组成，以抗双链DNA（72.55%）、抗Ro/SSA（89.8%）和抗La/SSB（45.88%）为主，除了肺部损伤较高（P = 0.017）外，没有特定的临床模式。我们的研究表明，在cSLE背景下，抗双链DNA与抗磷脂自身抗体的共存与抗磷脂综合征的发病率升高有关。这种关联与肾炎发生率的相应增加不一致。相反，抗双链DNA与抗Ro/SSA和抗La/SSB抗体的簇与肺部损伤有关，需要密切监测。
